Home | About us | Editorial board | Search | Ahead of print | Current issue | Archives | Submit article | Instructions | Subscribe | Contacts | Advertise | Login 
 
Search Article 
  
Advanced search 
  Users Online: 1027 Home Print this page Email this page Small font sizeDefault font sizeIncrease font size  

 
Table of Contents
REVIEW ARTICLE
Year : 2017  |  Volume : 21  |  Issue : 3  |  Page : 464-469

Endocrine glands and hearing: Auditory manifestations of various endocrine and metabolic conditions


1 Department of Endocrinology, Diabetes and Metabolism, Christian Medical College, Vellore, Tamil Nadu, India
2 Department of ENT, Christian Medical College, Vellore, Tamil Nadu, India

Date of Web Publication2-May-2017

Correspondence Address:
Thomas Vizhalil Paul
Department of Endocrinology, Diabetes and Metabolism, Christian Medical College, Vellore - 632 004, Tamil Nadu
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijem.IJEM_10_17

Rights and Permissions
   Abstract 

The aetiology of hearing loss in humans is multifactorial. Besides genetic, environmental and infectious causes, several endocrine and metabolic abnormalities are associated with varying degrees of hearing impairment. The pattern of hearing loss may be conductive, sensori-neural or mixed. The neurophysiology of hearing as well as the anatomical structure of the auditory system may be influenced by changes in the hormonal and metabolic milieu. Optimal management of these conditions requires the integrated efforts of the otolaryngologist and the endocrinologist. The presence of hearing loss especially in the young age group should prompt the clinician to explore the possibility of an associated endocrine or metabolic disorder for timely referral and early initiation of treatment.

Keywords: Endocrine systems, hearing loss, Pendred syndrome, Turner syndrome


How to cite this article:
Cherian KE, Kapoor N, Mathews SS, Paul TV. Endocrine glands and hearing: Auditory manifestations of various endocrine and metabolic conditions. Indian J Endocr Metab 2017;21:464-9

How to cite this URL:
Cherian KE, Kapoor N, Mathews SS, Paul TV. Endocrine glands and hearing: Auditory manifestations of various endocrine and metabolic conditions. Indian J Endocr Metab [serial online] 2017 [cited 2019 Nov 18];21:464-9. Available from: http://www.ijem.in/text.asp?2017/21/3/464/205474


   Introduction Top


Hearing loss is a common problem encountered in clinical practice. In addition to aging, various genetic and environmental factors play an important role in hearing loss. Some disorders and syndromes of endocrine system have shown to be associated with deafness. They may either cause or adversely influence diseases of the ear.

Diabetes mellitus, hypothyroidism, hypogonadism, acromegaly, osteogenesis imperfecta (OI), hypoparathyroidism, and Paget's disease of bone are some of the diseases which may have varying degrees of hearing abnormalities attributable to different mechanisms. This brief review attempts to describe the association of hearing impairment with disorders of various endocrine glands.


   Hearing and Hypothalamic-pituitary Disorders Top


Acromegaly

Acromegaly, a state of growth hormone (GH) excess due to a somatotroph adenoma, has been shown to be associated with hearing loss. There is an increased prevalence of both conductive and sensorineural deafness in patients with acromegaly. It has been suggested that deafness in acromegaly occurs because of bone hypertrophy and narrowing of internal auditory canals.[1] The mechanism of conductive hearing loss is postulated to be due to inadequate ventilation of the middle ear due to eustachian tube dysfunction as a result of redundant soft tissue and mucosa.[2] In addition, hearing loss may be caused by middle ear pressure dysregulation, sclerosis of mastoid, recurrent otitis, plaque formation, and GH excess associated increase in perilymph formation.[3]

Growth hormone deficiency

Hearing loss has been reported in mutations of genes encoding insulin-like growth factor 1 (IGF 1) and the GH receptor. IGF 1 and its receptor are expressed abundantly in the central nervous system, including the inner ear. Being the chief effector molecule of GH, IGF 1 is required for the differentiation of neurons and the maturation of inner ear cells. IGF 1 influences hearing by protecting hair cells from undergoing apoptosis, suppressing the downregulation of proapoptotic genes and regulating glucose transporters of the outer hair cells, thereby promoting neuronal survival.[4] The prevalence of misophonia (reduced sound tolerance) and high-tone sensorineural hearing loss was shown to be more in individuals with untreated congenital isolated GH deficiency than in controls. Misophonia may be related to changes in the limbic and autonomic nervous system and an absence of the stapedial reflex secondary to a reduction in depth of skull and facial height.[5]

Neurohypophyseal diabetes insipidus

Diabetes insipidus, diabetes mellitus, optic atrophy, and deafness DIDMOAD syndrome is a rare autosomal recessive disorder caused by mutations in the wolframin (WFS 1) gene which is expressed in pancreatic beta cells, neurons, and neuroendocrine tissues. Although diabetes mellitus and optic atrophy are the defining features of this complex disorder, diabetes insipidus occurs in about 70% and sensorineural deafness in about two-thirds of all patients. WFS 1 gene on chromosome 4p encodes a calcium channel that is responsible for maintaining the homeostasis of the endoplasmic reticulum (ER). Mutated wolframin forms intracellular aggregates [6] and contributes to unresolvable ER stress leading to apoptosis of beta cells and neurons.[7]

Craniopharyngioma

Although craniopharyngiomas are usually confined to the sellar-suprasellar location, a subgroup of giant craniopharyngioma may extend into the posterior cranial fossa. They account for approximately 4% of cases, and can present with unilateral or bilateral hearing loss as the initial symptom, before other neurological features become evident. Children who present with deafness should be referred to hearing specialists early and language rehabilitation should be initiated as hearing loss can compromise language development and cognitive function.[8]

Sellar/ectopic pituitary adenoma

Hearing impairment caused by pituitary adenomas has been described in literature.[9],[10],[11] Often, these have been described in invasive prolactin-secreting adenomas of sellar origin, wherein direct extension and involvement of the cerebellopontine angle instead of the bony internal auditory canal is implicated as the cause of hearing loss.[9],[11] Ectopic pituitary adenomas may also present with hearing loss due to compression of the internal auditory canal.[12]


   Hearing and Thyroid-parathyroid Disorders Top


Congenital hypothyroidism (nongenetic)

Endemic cretinism occurs due to severe iodine deficiency over several generations and is invariably associated with goiter. In North India, the prevalence of endemic cretinism was reported to be 3%–5% and deaf-mutism was seen in about three-fourths of them.[13],[14]

The causes for auditory abnormalities in congenital hypothyroidism include the following:[15]
  1. Increased connective tissue and osseous changes throughout the labyrinth
  2. Alterations in the petrous temporal bone and ectatic changes in the semicircular canals and cochlea
  3. Atrophy of the stria vascularis
  4. Degeneration of spiral ganglion and hair cells of the organ of Corti
  5. Generalized atrophy of organ of Corti
  6. Incomplete ossification of the stapes and anomalies of incus and malleus
  7. Distortion of oval and round windows
  8. The presence of hyaline streak between the organ of Corti and tectorial membrane.


Congenital hypothyroidism (genetic)

The syndrome of sporadic goiter and hearing loss with slow development of speech was described by Vaughn Pendred in 1896. Eponymously known as Pendred syndrome (PDS), this autosomal recessive disorder is characterized by iodine organification defects, presence of goiter, sensorineural hearing loss, and a positive perchlorate discharge test. It may account for 5%–10% of all cases of congenital deafness. The hearing loss is more pronounced at higher frequencies. PDS is caused by mutations of the SLC26A4 gene on chromosome 7q31 that encodes pendrin, which is a transmembrane exchanger of chloride, iodide, and bicarbonate ions and is expressed in the thyroid, inner ear, and kidney.[16] The sensorineural deafness is usually associated with malformation of the cochlea which is hypoplastic and known as “Mondini cochlea.”[17]

Acquired hypothyroidism

The etiology of hearing loss in hypothyroidism can be conductive, sensorineural, or mixed and has been documented to occur in more than half of the individuals.[18] The conductive hearing loss in these patients is probably due to reduced compliance because of hypertrophy and edema of the mucosa of the nose and eustachian tube leading to eustachian obstruction and also related to thickening of the tympanic membrane. Other postulated mechanisms include changes in ossicles such as distortion of incus and stapes, and obstruction of round or oval windows.[19] Sensorineural deafness has been attributed to changes in the cochlea probably secondary to accumulation of acid mucopolysaccharides and altered nerve conduction.[18]

Resistance to thyroid hormones (Refetoff syndrome)

It has been shown that thyroid hormones and their receptors are involved in the auditory system through multiple pathways. Thyroid hormone beta receptors are expressed in the cochlea and are involved in auditory development. Refetoff syndrome is characterized by mutations in the gene encoding the beta receptor and an inability of the target tissues to take up thyroid hormones resulting in high circulating levels of thyroid hormone and high urinary iodide levels. Deafness has been reported in several patients with resistance to thyroid hormone.[19] Although some patients are asymptomatic, others may present with goiter, tachycardia, and hyperactivity.

Graves' disease and antithyroid drugs

Although hearing loss is not commonly seen in association with Graves' disease, decreased hearing ability particularly at higher frequencies has occasionally been reported.[20] Exact mechanisms are yet to be elucidated. There have also been reports of ototoxicity secondary to ANCA-positive vasculitis in patients treated with propylthiouracil, which manifests as sensorineural hearing loss.[21]

Hypoparathyroidism

Barakat syndrome, also known as hypoparathyroidism, deafness, and renal dysplasia (HDR) syndrome, is an autosomal dominant disorder characterized by hypoparathyroidism, sensorineural deafness, and renal dysplasia. Mutations in the GATA 3 gene located on the short arm of chromosome 10 (10p) result in the premature termination of the GATA 3 protein with loss of basic amino acids in the flanking ZnF2 domain.[22] Although widely expressed in the central and peripheral nervous system, parathyroid glands, inner ear, and kidneys are the most commonly affected organs. Sensorineural deafness occurs early and is the most penetrant aspect of the HDR syndrome. The hearing loss is secondary to involvement of peripheral part of the auditory system and the absence of otoacoustic emissions and is more pronounced at higher frequencies.[23]

Hyperparathyroidism

Deafness is not commonly encountered in hyperparathyroidism; however, a review of literature revealed occasional reports of hearing loss and aphonia in association with hyperparathyroidism. Calcification of the ear drums were noticed in these cases and were found to resolve after parathyroidectomy with complete or partial reversal of hearing loss.[24]


   Hearing Loss and Metabolic Bone Disease Top


Paget's disease of bone

Hearing loss is long recognized as a complication of Paget's disease. Previously, various mechanisms were proposed to cause hearing impairment and included loss of auditory hair cells and ganglion cells, stretching of the auditory nerve and toxic cytokines.[25] Thereafter, studies done by Khetarpal and Schuknecht on the histological appearance of pagetic temporal bone inferred that both conductive and sensory hearing loss are caused by changes in the bone density, mass and form, resulting in the dampening of the motion mechanics of the middle and inner ears.[26]

Osteogenesis imperfecta

With an estimated prevalence of 1 in 10,000–20,000 births, OI is one of the most commonly inherited bone diseases. It occurs due to defects in the synthesis of type 1 collagen or its intracellular processing and manifests as an increased susceptibility to fractures, bony deformities, joint laxity, blue sclerae, dentinogenesis imperfecta, and hearing loss which can be conductive, sensorineural, or mixed.[27],[28] In population studies, the estimated prevalence of OI-associated hearing loss is 45%–58%.[29] The mechanisms involved in the pathogenesis of deafness in OI are manifold and include the following:

  1. Atrophy of cochlear hair cells and stria vascularis
  2. Abnormal bone formation in the cochlea and surrounding structures
  3. Footplate fixation and deficient ossification of the ossicles
  4. Atrophy or fractures of stapes or malleus
  5. Mucosal hypervascularization or otospongiosis-like lesions in the stapes footplate causing fixation or discontinuity of the ossicular chain.


Although the conductive hearing loss caused by ossicular deformities can often be surgically corrected, outcomes are worse than in patients without OI probably due to diminished stability of the supporting bones and possible thickening and hypervascularization of the footplate.[30]

Fibrous dysplasia

Fibrous dysplasia is a slow-growing condition of the bone, in which there is progressive replacement with fibrous tissue and disorganized bony trabeculae. The temporal bone is rarely involved in monostotic forms of the disease resulting in narrowing of the external auditory meatus, otalgia, recurrent otitis media, and formation of secondary cholesteatoma leading to hearing loss.[31]

Hereditary hypophosphatemic osteomalacia

This forms a heterogeneous group of disorders characterized by renal phosphate wasting. They occur due to mutations in various genes such as PHEX (phosphate-regulating gene with homologies to endopeptidase on X chromosome) that causes X-linked hypophosphatemic rickets (XLH), fibroblast growth factor (FGF) 23, causing autosomal dominant hypophosphatemic rickets, dentin matrix protein (DMP) causing autosomal recessive hypophosphatemic rickets (ARHR) type 1, and ectonucleotide pyrophosphatase (ENPP) causing ARHR type 2.

Conductive hearing loss has been reported to occur in PHEX gene mutations. Mutations involving inactivation of DMP 1 have been associated with sensorineural hearing loss due to narrowing of the internal auditory canal. ENPP mutation has been reported to cause a mixed hearing loss, initially conductive and then sensorineural.[32] In XLH, the mechanism underlying deafness appears to be involvement of the cochlea, secondary to osteosclerosis of the petrous temporal bone.

Osteoporosis

A recent meta-analysis of five studies showed a significant association between decrease in bone mineral density (BMD) and hearing loss. There was a statistically significant increased odds of hearing loss in the low BMD or osteoporosis group with OR of 1.20.[33] Osteoporosis is associated with demineralization of the skeleton and involvement of the temporal bone that contains the cochlear capsule, is considered to be responsible for the hearing loss.

Inherited sclerotic bone disorders

Most inherited sclerosing bone dysplasias present with cranial nerve dysfunction due to compression of the narrow foramina that carry them. Among these Voorhoeve's disease is classically described to present with hearing loss.[34]


   Gonadal Disorders and Hearing Loss Top


Hypogonadotropic hypogonadism

A clinical diagnosis of Kallman's syndrome is made in the presence of hypogonadism and anosmia. Other manifestations include renal and dental agenesis, cleft lip and palate, mirror movements, and hearing loss. Deafness has been attributed to several genetic mutations in KAL1, fibroblast growth factor receptor 1, FGF 8, interleukin 17 receptor D, CHD7, and the transcriptional factor SOX 10 that regulate the development of neural crest cells.[35]

Coloboma, heart defects, choanal atresia, retardation of growth and development, genital abnormalities, and ear abnormalities (CHARGE) syndrome caused by mutations in CHD7 can present similarly with hypogonadism, anosmia, cleft palate, and hearing loss. CHARGE syndrome is associated with distortion of the semicircular canals.[35] Hearing loss is due to many factors such as ossicular malformation, eustachian tube dysfunction, otitis media secondary to craniofacial anomalies, dysplastic cochlea, and reduced diameter of auditory nerve.[36]

Hypergonadotropic hypogonadism

Turner syndrome (TS) is characterized by short stature and gonadal dysgenesis secondary to loss of one of the X chromosomes in affected females. Hearing loss has been reported in women with TS and can be conductive, sensorineural, or mixed. It occurs in young and middle-aged women and tends to be progressive with advancing age.

Recurrent otitis media occurs due to abnormal eustachian tube structure and function that leads to scarring of the tympanic membrane causing conductive hearing loss. Other mechanisms of conductive hearing loss include ossicular necrosis and cholesteatoma in the middle ear. Anatomical malformation of the skull base resulting in poor drainage and inadequate ventilation of the middle ear also contribute to middle ear involvement. Lymphatic hypoplasia resulting in persistent lymphatic effusion also causes impaired aeration and drainage.

The patterns of sensorineural deafness include a mid-frequency dip and a high-frequency loss. The etiology of sensorineural hearing loss is thought to be an X-linked dosage effect resulting in fewer sensory cells in the cochlea leading to cochlear dysfunction. Estrogen deficiency-associated poor mineralization of the cochlear capsule and its role in hearing impairment has not been well studied.[37]


   Adrenal Disorders Top


Adrenoleukodystrophy is a rare X-linked condition that is caused by mutations in the adenosine triphosphate binding cassette group of transporter genes. The gene encodes the enzyme fatty acylCoA ligase which is responsible for the peroxisomal transfer of fatty acids. Defective enzymatic activity leads to prevention of transport of fatty acids into the peroxisomes and their accumulation within cells.

Clinical manifestations include behavioral abnormalities, vision and hearing deficits, abnormalities in speech, gait, writing, memory, and adrenal insufficiency. In advanced cases, motor and cognitive dysfunction, dysphagia, and convulsions may occur. Hearing impairment is secondary to deficient auditory processing in the presence of demyelination involving the auditory and other parts of the brain.[38]


   Diabetes Mellitus and Hearing Loss Top


Type 2 diabetes mellitus has been reported to be associated with microvascular complications that may affect hearing. Duration of diabetes and poor glycemic control has been linked to sensorineural hearing loss after adjusting for age.[39] Basement membrane thickening of the vascular endothelium is one of the most prevalent morphologic findings in diabetes mellitus. The proposed mechanisms implicated in the causation of hearing loss are as follows:

  1. Diabetic angiopathy causing an interruption in cochlear blood supply and transportation of nutrients
  2. Vasculopathy that leads to secondary degeneration of the eighth nerve
  3. Thickening of the capillaries of the stria vascularis
  4. Atrophy of the spiral ganglion
  5. Demyelination of the eighth nerve
  6. Perineurial fibrosis
  7. Decreased nerve fiber density in the spiral lamina
  8. Reduction in number of cells in the middle and basal turns of the cochlea
  9. Reduced ganglion cells in the dorsal and ventral cochlear nuclei
  10. Reduced ganglion cells in the superior olivary complex, inferior colliculus, and medial geniculate body.[40]



   Miscellaneous Causes Top


Mitochondrial deafness

Mutations in mitochondrial DNA may result in deafness. As mitochondria are required for oxidative phosphorylation, mitochondrial dysfunction usually results in pleiotropic effects affecting different organ systems.[41]

Kearns–Sayre syndrome

This occurs due to large-scale mitochondrial DNA rearrangements and manifests as chronic progressive external ophthalmoplegia, diabetes, retinitis pigmentosa, cardiac conduction defects, anemia, hypoparathyroidism, and deafness.

Maternally inherited diabetes and deafness

Maternally inherited diabetes and deafness is a mitochondrial disorder, transmitted in a dominant fashion in which an affected mother transmits the disease to all her offspring. An affected father does not transmit the disease to his progeny as the mitochondria contained in the sperm are shed before the entry of the sperm nucleus into the egg. Diabetes occurs due to pancreatic beta cell dysfunction and progressive insulinopenia. Sensorineural hearing loss is bilateral, more common in men and is considered to be due to atrophy of cochlear stria vascularis. Rarely other features such as GH and adrenocorticotropic hormone deficiency, hypothalamic hypogonadism, secondary hypothyroidism, and cardiomyopathy may occur.[42]

Familial paragangliomas and hearing loss

Carotid body paragangliomas are extremely rare tumors that may often be familial; their genetic inheritance is through paternal imprinting. There are families reported to have inherited paragangliomas with sensorineural deafness.[43] In addition, paragangliomas of tympanic and vagal origin are known to present with tinnitus and hearing loss as early symptoms.[44]


   Conclusion Top


Hearing disorders may be a part of various endocrine and metabolic conditions. Early identification and timely referral to hearing specialists are of utmost importance, especially in children, as hearing loss can have a bearing on language development. Otolaryngologists should also be abreast of the hearing disorders that manifests along with endocrine conditions. A high index of suspicion needs to be maintained to identify hearing loss that occurs as part of syndromes or states of hormone excess or deficiency.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
   References Top

1.
Richards S. Deafness in acromegaly. J Laryngol Otol 1968;82:1053-65.  Back to cited text no. 1
[PUBMED]    
2.
Kuan EC, Peng KA, Kita AE, Bergsneider M, Wang MB. Acromegaly: Otolaryngic manifestations following pituitary surgery. Am J Otolaryngol 2015;36:521-5.  Back to cited text no. 2
    
3.
Aydin K, Ozturk B, Turkyilmaz MD, Dagdelen S, Ozgen B, Unal F, et al. Functional and structural evaluation of hearing in acromegaly. Clin Endocrinol (Oxf) 2012;76:415-9.  Back to cited text no. 3
[PUBMED]    
4.
Varela-Nieto I, Morales-Garcia JA, Vigil P, Diaz-Casares A, Gorospe I, Sánchez-Galiano S, et al. Trophic effects of insulin-like growth factor-I (IGF-I) in the inner ear. Hear Res 2004;196:19-25.  Back to cited text no. 4
    
5.
Prado-Barreto VM, Salvatori R, Santos Júnior RC, Brandão-Martins MB, Correa EA, Garcez FB, et al. Hearing status in adult individuals with lifetime, untreated isolated growth hormone deficiency. Otolaryngol Head Neck Surg 2014;150:464-71.  Back to cited text no. 5
    
6.
Rutishauser J, Spiess M, Kopp P. Genetic forms of neurohypophyseal diabetes insipidus. Best Pract Res Clin Endocrinol Metab 2016;30:249-62.  Back to cited text no. 6
[PUBMED]    
7.
Fonseca SG, Ishigaki S, Oslowski CM, Lu S, Lipson KL, Ghosh R, et al. Wolfram syndrome 1 gene negatively regulates ER stress signaling in rodent and human cells. J Clin Invest 2010;120:744-55.  Back to cited text no. 7
[PUBMED]    
8.
Connolly ES Jr., Winfree CJ, Carmel PW. Giant posterior fossa cystic craniopharyngiomas presenting with hearing loss. Report of three cases and review of the literature. Surg Neurol 1997;47:291-9.  Back to cited text no. 8
    
9.
Ahmed M, Al-Nozha O. Images in clinical medicine. Large prolactinoma. N Engl J Med 2010;363:177.  Back to cited text no. 9
[PUBMED]    
10.
Singh NP, Panwar VK. Case report of a pituitary macroadenoma treated with artemether. Integr Cancer Ther 2006;5:391-4.  Back to cited text no. 10
[PUBMED]    
11.
Schöfl C, Schöfl-Siegert B, Karstens JH, Bremer M, Lenarz T, Cuarezma JS, et al. Falsely low serum prolactin in two cases of invasive macroprolactinoma. Pituitary 2002;5:261-5.  Back to cited text no. 11
    
12.
Liang J, Libien J, Kunam V, Shao C, Rao C. Ectopic pituitary adenoma associated with an empty sella presenting with hearing loss: Case report with literature review. Clin Neuropathol 2014;33:197-202.  Back to cited text no. 12
    
13.
Sankar R, Pulger T, Rai B, Gomathi S, Gyatso TR, Pandav CS. Epidemiology of endemic cretinism in Sikkim, India. Indian J Pediatr 1998;65:303-9.  Back to cited text no. 13
    
14.
Kapil U, Jain V, Kabra M, Pandey RM, Sareen N, Khenduja P. Prevalence of neonatal hypothyroidism in Kangra Valley, Himachal Pradesh. Eur J Clin Nutr 2014;68:748-9.  Back to cited text no. 14
    
15.
Meyerhoff EL. The thyroid and audition. Laryngoscope 1976;86:483-9.  Back to cited text no. 15
    
16.
Anwar S, Riazuddin S, Ahmed ZM, Tasneem S, Ateeq-ul-Jaleel, Khan SY, et al. SLC26A4 mutation spectrum associated with DFNB4 deafness and Pendred's syndrome in Pakistanis. J Hum Genet 2009;54:266-70.  Back to cited text no. 16
    
17.
Pearce JM. Pendred's syndrome. Eur Neurol 2007;58:189-90.  Back to cited text no. 17
    
18.
Malik V, Shukla GK, Bhatia N. Hearing profile in hypothyroidism. Indian J Otolaryngol Head Neck Surg 2002;54:285-90.  Back to cited text no. 18
    
19.
Melse-Boonstra A, Mackenzie I. Iodine deficiency, thyroid function and hearing deficit: A review. Nutr Res Rev 2013;26:110-7.  Back to cited text no. 19
    
20.
Berker D, Karabulut H, Isik S, Tutuncu Y, Ozuguz U, Erden G, et al. Evaluation of hearing loss in patients with Graves' disease. Endocrine 2012;41:116-21.  Back to cited text no. 20
    
21.
Thamprajamchit S, Jariengprasert C, Rajatanavin R. Propylthiouracil-induced sensorineural hearing loss associated with antineutrophil cytoplasmic antibodies. Endocr Pract 2004;10:432-7.  Back to cited text no. 21
    
22.
Döneray H, Usui T, Kaya A, Dönmez AS. The first Turkish case of hypoparathyroidism, deafness and renal dysplasia (HDR) syndrome. J Clin Res Pediatr Endocrinol 2015;7:140-3.  Back to cited text no. 22
    
23.
van Looij MA, Meijers-Heijboer H, Beetz R, Thakker RV, Christie PT, Feenstra LW, et al. Characteristics of hearing loss in HDR (hypoparathyroidism, sensorineural deafness, renal dysplasia) syndrome. Audiol Neurootol 2006;11:373-9.  Back to cited text no. 23
    
24.
Simpson JA. Aphonia and deafness in hyperparathyroidism. Br Med J 1954;1:494-6.  Back to cited text no. 24
    
25.
Monsell EM. The mechanism of hearing loss in Paget's disease of bone. Laryngoscope 2004;114:598-606.  Back to cited text no. 25
    
26.
Khetarpal U, Schuknecht HF. In search of pathologic correlates for hearing loss and vertigo in Paget's disease. A clinical and histopathologic study of 26 temporal bones. Ann Otol Rhinol Laryngol Suppl 1990;145:1-16.  Back to cited text no. 26
    
27.
Cheung MS, Glorieux FH. Osteogenesis imperfecta: Update on presentation and management. Rev Endocr Metab Disord 2008;9:153-60.  Back to cited text no. 27
    
28.
Pillion JP, Vernick D, Shapiro J. Hearing loss in osteogenesis imperfecta: Characteristics and treatment considerations. Genet Res Int 2011;2011:983942.  Back to cited text no. 28
    
29.
Swinnen FK, Coucke PJ, De Paepe AM, Symoens S, Malfait F, Gentile FV, et al. Osteogenesis imperfecta: The audiological phenotype lacks correlation with the genotype. Orphanet J Rare Dis 2011;6:88.  Back to cited text no. 29
    
30.
Pereira da Silva A, Feliciano T, Figueirinhas R, Almeida E Sousa C. Osteogenesis imperfecta and hearing loss – description of three case reports. Acta Otorrinolaringol Esp 2013;64:423-7.  Back to cited text no. 30
    
31.
Falcioni M, De Donato G. Fibrous dysplasia of the temporal bone. Am J Otol 2000;21:887-8.  Back to cited text no. 31
    
32.
Steichen-Gersdorf E, Lorenz-Depiereux B, Strom TM, Shaw NJ. Early onset hearing loss in autosomal recessive hypophosphatemic rickets caused by loss of function mutation in ENPP1. J Pediatr Endocrinol Metab 2015;28:967-70.  Back to cited text no. 32
    
33.
Upala S, Rattanawong P, Vutthikraivit W, Sanguankeo A. Significant association between osteoporosis and hearing loss: A systematic review and meta-analysis. Braz J Otorhinolaryngol 2016. pii: S1808-869430176-8.  Back to cited text no. 33
    
34.
Odrezin GT, Krasikov N. CT of the temporal bone in a patient with osteopathia striata and cranial sclerosis. AJNR Am J Neuroradiol 1993;14:72-5.  Back to cited text no. 34
    
35.
Salama N. Kallmann syndrome and deafness: An uncommon combination: A case report and a literature review. Int J Reprod Biomed (Yazd) 2016;14:541-4.  Back to cited text no. 35
    
36.
Thelin JW, Mitchell JA, Hefner MA, Davenport SL. CHARGE syndrome. Part II. Hearing loss. Int J Pediatr Otorhinolaryngol 1986;12:145-63.  Back to cited text no. 36
    
37.
Alves C, Oliveira CS. Hearing loss among patients with Turner's syndrome: Literature review. Braz J Otorhinolaryngol 2014;80:257-63.  Back to cited text no. 37
    
38.
Bamiou DE, Davies R, Jones S, Musiek FE, Rudge P, Stevens J, et al. An unusual case of X-linked adrenoleukodystrophy with auditory processing difficulties as the first and sole clinical manifestation. J Am Acad Audiol 2004;15:152-60.  Back to cited text no. 38
    
39.
Mitchell P, Gopinath B, McMahon CM, Rochtchina E, Wang JJ, Boyages SC, et al. Relationship of type 2 diabetes to the prevalence, incidence and progression of age-related hearing loss. Diabet Med 2009;26:483-8.  Back to cited text no. 39
    
40.
Diniz TH, Guida HL. Hearing loss in patients with diabetes mellitus. Braz J Otorhinolaryngol 2009;75:573-8.  Back to cited text no. 40
    
41.
Kokotas H, Petersen MB, Willems PJ. Mitochondrial deafness. Clin Genet 2007;71:379-91.  Back to cited text no. 41
    
42.
Naing A, Kenchaiah M, Krishnan B, Mir F, Charnley A, Egan C, et al. Maternally inherited diabetes and deafness (MIDD): Diagnosis and management. J Diabetes Complications 2014;28:542-6.  Back to cited text no. 42
    
43.
Lord RS, Chambers AJ. Familial carotid body paragangliomas and sensorineural hearing-loss: A new syndrome. Cardiovasc Surg 1999;7:134-8.  Back to cited text no. 43
    
44.
Apple D, Kreines K. Cushing's syndrome due to ectopic ACTH production by a nasal paraganglioma. Am J Med Sci 1982;283:32-5.  Back to cited text no. 44
    



This article has been cited by
1 FGF23 and its role in X-linked hypophosphatemia-related morbidity
Signe Sparre Beck-Nielsen,Zulf Mughal,Dieter Haffner,Ola Nilsson,Elena Levtchenko,Gema Ariceta,Carmen de Lucas Collantes,Dirk Schnabel,Ravi Jandhyala,Outi Mäkitie
Orphanet Journal of Rare Diseases. 2019; 14(1)
[Pubmed] | [DOI]



 

Top
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

   Abstract Introduction Hearing and Hypo... Hearing and Thyr... Hearing Loss and... Gonadal Disorder... Adrenal Disorders Diabetes Mellitu... Miscellaneous Causes Conclusion
  In this article
 References

 Article Access Statistics
    Viewed1293    
    Printed9    
    Emailed0    
    PDF Downloaded296    
    Comments [Add]    
    Cited by others 1    

Recommend this journal