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Causes of Hearing Loss, with ICD-10 codes

The following list is offered for perspective on the myriad associations to consider when caring for a child with persistent hearing impairment, particularly when the etiology is, as yet, unknown. Information was compiled from Orphanet, Online Mendelian Inheritance in Man (OMIM), and [Toriello: 2013] and previous editions.

Several of the !CD-10 codes are not specific to the condition named but subsume that condition and others. Some codes listed will need an extra digit or two (indicated by "x" and "xx") to provide optimal specificity (for location, timing, severity, visit type, etc.) – please consult icd10data.com, another ICD-10 reference, or a coding specialist if you intend to use these for billing purposes. No ICD-10 code is listed when there are multiple options for coding or we couldn't find one (if you know the code, please use the Feedback button at the top of the page to let us know).
ICD-10 Cause
Autosomal dominant inheritance
Q77.4 Achondroplasia
Q87.0 Apert syndrome
H90.3 Autosomal dominant nonsyndromic sensorineural deafness type, DFNA (many genes)
H47.2 Autosomal dominant optic atrophy and congenital deafness syndrome
H47.2 Autosomal dominant optic atrophy plus syndrome (deafness - polyneuropathy - myopathy)
Q87.0 Branchio-oto-renal syndrome
Q87.3 Camurati-Engelmann syndrome
Q89.8 CHARGE association (Coloboma, Heart defect, Atresia choanae, Retarded growth and development, Genital hypoplasia, Ear anomalies/deafness)
Q74.0 Cleidocranial dysostosis
Q87.0 Craniofacial-deafness-hand syndrome
Q75.1 Crouzon disease (craniofacial dysostosis)
H90.3 Dominant nonsyndromic hearing loss
H50.81x Duane syndrome (found in cervico-oculo-acoustic dysplasia or Wildervanck syndrome and in HOXA-1-related syndromes, including Bosley-Salih-Alorainy syndrome and Athabaskan brain stem dysgenesis syndrome)
D61.09 Fanconi anemia
Q75.8 Hemifacial microsomia
Q78.8 Hermann syndrome (multiple synostoses syndrome)
Q87.89 LEOPARD syndrome (Lentigines, ECG conduction abnormalities, Ocular hypertelorism, Pulmonic stenosis, Abnormal genitalia, Retardation of growth, and sensorineural Deafness)
Q87.0 Möbius syndrome (oculo-facial paralysis)
M04.2 Muckle-Wells syndrome
Q78.8 Multiple synostoses syndrome
Q85.02 Neurofibromatosis, type 2 (NF2)
H80.90 Otosclerosis (* familial form)
M88 Paget disease (juvenile)
E70.39 Piebaldism
Q82.4 Robinson-type ectodermal dysplasia
Q82.8 Schwann syndrome, aka Bart-Pumphrey syndrome
Q87.0 Stickler syndrome
Q75.4 Treacher Collins syndrome
M85.2 Van Buchem syndrome
E70.8 Waardenburg syndrome, types 1-4
Thought to involve Autosomal dominant inheritance
Q87.0 Baraitser-Winter (Iris coloboma - ptosis - intellectual deficit) syndrome
Q16.1 Congenital atresia of the external auditory canal
Q82.8 Vohwinkel-Nockemann syndrome
Q87.8 Wildervanck (cervico-oculo-acoustic) syndrome
Autosomal recessive inheritance
H90.5 Autosomal recessive nonsyndromic sensorineural hearing loss
D81.810 Biotinidase deficiency
Q78.8 Björnstad syndrome (deafness - pili torti - hypogonadism)
Carraro syndrome (absence of the tibia with congenital deafness)
Q87.1 Cockayne syndrome
Q77.5 Diastrophic dwarfism
Q87.0 Fraser syndrome
G11.1 Friedreich ataxia
H35.5 Hallgren syndrome, aka Usher syndrome
H18.5 Harboyan syndrome (corneal dystrophy - perceptive deafness)
E76.02 Hurler syndrome, aka Mucopolysaccharidosis Type I (MPS 1)
I45.8 Jervell and Lange-Nielsen syndrome
Q89.3 Kartagener syndrome (primary ciliary dyskinesia)
E76.210 Morquio syndrome (mucopolysaccharidosis type 4)
E70.329 Oculocutaneous albinism
E07.1 Pendred syndrome
Q78.5 Pyle disease
G60.1 Refsum disease
G60.2 Richards-Rundle syndrome
G60.8 Sensory radicular neuropathy
H35.5 Usher syndrome, types 1-3
E13.8 Wolfram syndrome
Thought to involve Autosomal recessive inheritance
Q87.0 Mohr syndrome (oral-facial-digital syndrome type 2)
G40.B11 Myocolonic epilepsy
Male-lethal X-linked inheritance
Q87.0 Oral-Facial-Digital syndrome
Mitochondrial inheritance
G71.3 Myoclonic epilepsy with ragged-red fibers (MERRF)
Sporadic occurence
Q90.9 Down Syndrome
Q91.7 Trisomy 13
Q91.3 Trisomy 18
Q96.9 Turner Syndrome
X-linked dominant inheritance
Q78.5 Fronto-metaphyseal dysplasia
Q78.0 Otopalatodigital syndrome (aka Taybi oto-palato-digital syndrome)
X-linked recessive inheritance
E76.1 Hunter syndrome, aka Mucopolysaccharidosis type II
Mohr-Tranebjaerg Syndrome (deafness-dystonia-optic neuronopathy syndrome)
H35.5 Norrie disease
H90.8 X-linked nonsyndromic hearing loss, DFNX
Genetic heterogeneity: Autosomal dominant, Autosomal recessive
Q87.0 Marshall syndrome
G71.0 Muscular dystrophy, including Myotonic Muscular Dystrophy Type 1
Q78.0 Osteogenesis imperfecta
Genetic heterogeneity : Autosomal dominant, Sporadic
Q87.0 Goldenhar syndrome
Genetic heterogeneity: Autosomal dominant, X-linked recessive
Q87.1 Cornelia de Lange Syndrome
Genetic heterogeneity: Autosomal dominant, Autosomal recessive, X-linked recessive
Q87.81 Alport syndrome
Q78.2 Osteopetrosis
Genetic heterogeneity: Autosomal dominant, Autosomal recessive, Sporadic
Q76.1 Klippel-Feil syndrome
Genetic heterogeneity: Autosomal recessive, Sporadic
Q87.0 Pierre Robin sequence
Congenitally acquired causes
G80.4 Cerebral Palsy
P35.1 Congenital cytomegalovirus infection
Q86.0 Fetal alcohol syndrome
E00.x Fetal iodine deficiency effects
T56.1 Fetal methyl mercury effects
Infectious causes
H66.xxx Otitis media with effusion, chronic
B05.89 Measles
Meningitis (viral or bacterial)
B26.89 Mumps
A38.89 Scarlet fever
P00.2 TORCH infections (Toxoplasmosis, Other, Rubella, Cytomegalovirus, Herpes simplex virus type 2; Other can include cosxsackieviurs, syphilis, varicella-zoster virus, HIV, parvovirus B19)
Traumatic causes
S09.20Xx Acoustic trauma, such as from explosions, fireworks, gunfire, rock concerts, and earphones
S09.20Xx Barotrauma (differences in pressure)
S09.19Xx Skull fracture (temporal bone)
S09.20Xx Traumatic perforation of the eardrum
Toxic causes
T39.015x Aspirin and other salicylates
T36.5X5x Aminoglycoside antibiotics
T45.1X5x Cisplatin
T45.1X5x Carboplatin
T36.2X1x Dichloromethotrexate (DCM)
T42.0X1x Diflouromethylornithine (DFMO)
T50.905x Erythromycin
T45.2X1x Ethacrynic acid
T50.1X5x Loop diuretics
T42.71Xx Misonidazole
T53.5X1x Nitrogen mustard
T39.391x NSAID
T45.511x Quinine and quinine derivatives
T36.95Xx Vancomycin
T42.1X5x Vincristine
Other causes
D33.3 Acoustic neuroma
H81.09 Meniere disease
P07.xx Complications of very early prematurity/low birth weight
H72.9x Persistent perforation of ear drum

Authors & Reviewers

Initial publication: August 2013; last update/revision: August 2013
Current Authors and Reviewers:
Reviewer: Chuck Norlin, MD
Authoring history
2013: revision: John C. Carey, MDR
2002: first version: Daryl Scott, MD, Ph.D.A
AAuthor; CAContributing Author; SASenior Author; RReviewer

Page Bibliography

Toriello HV, Smith SD.
Hereditary Hearing Loss and Its Syndromes.
3rd ed. Oxford: Oxford University Press; 2013. 978-0-19-973196-1