List of Causes of Hearing Loss with ICD-9 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.

Most of the ICD-9 codes listed will need an extra digit or two to provide optimal specificity (for location, timing, severity, etc.) – please consult an ICD-9 reference [ICD-9-CM: 2013] or coding specialist if you intend to use these for billing purposes. No ICD-9 code is listed when there are multiple options for coding, no specific code exists, 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-9 Cause
Autosomal dominant inheritance
756.4 Achondroplasia
755.55 Apert syndrome
H90.3 Autosomal dominant nonsyndromic sensorineural deafness type, DFNA (many genes)
Autosomal dominant optic atrophy and congenital deafness syndrome
Autosomal dominant optic atrophy plus syndrome (deafness - polyneuropathy - myopathy)
Branchio-oto-renal syndrome
756.59 Camurati-Engelmann syndrome
759.9 CHARGE association (Coloboma, Heart defect, Atresia choanae, Retarded growth and development, Genital hypoplasia, Ear anomalies/deafness)
755.59 Cleidocranial dysostosis
Craniofacial-deafness-hand syndrome
756 Crouzon disease (craniofacial dysostosis)
Dominant nonsyndromic hearing loss
378.71 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)
284.09 Fanconi Anemia
754.0 Hemifacial microsomia
Hermann syndrome (multiple synostoses syndrome)
LEOPARD syndrome (Lentigines, ECG conduction abnormalities, Ocular hypertelorism, Pulmonic stenosis, Abnormal genitalia, Retardation of growth, and sensorineural Deafness)
352.6 Möbius syndrome (oculo-facial paralysis)
Muckle-Wells syndrome
755.8 Multiple synostoses syndrome
237.72 Neurofibromatosis, type 2 (NF2)
387 Otosclerosis (* familial form)
731.0 Paget disease (juvenile)
709.09 Piebaldism
757.31 Robinson type ectodermal dysplasia
Schwann syndrome, aka Bart-Pumphrey syndrome
759.89 Stickler syndrome
756.0 Treacher Collins syndrome
733.3 Van Buchem syndrome
756.89 Waardenburg syndrome, types 1-4
Thought to involve Autosomal dominant inheritance
Baraitser-Winter (Iris coloboma - ptosis - intellectual deficit) syndrome
744.02 Congenital atresia of the external auditory canal
Vohwinkel-Nockemann syndrome
759.89 Wildervanck (cervico-oculo-acoustic) syndrome
Autosomal recessive inheritance
Autosomal recessive nonsyndromic sensorineural hearing loss
277.6 Biotinidase deficiency
757.4 Björnstad syndrome (deafness - pili torti - hypogonadism)
Carraro syndrome (absence of the tibia with congenital deafness)
759.89 Cockayne syndrome
Diastrophic dwarfism
759.89 Fraser syndrome
334 Friedreich ataxia
Hallgren syndrome, aka Usher syndrome
Harboyan syndrome (corneal dystrophy - perceptive deafness)
277.5 Hurler syndrome, aka Mucopolysaccharidosis Type I OLD
426.82 Jervell and Lange-Nielsen syndrome
759.3 Kartagener syndrome (primary ciliary dyskinesia)
277.5 Morquio syndrome (mucopolysaccharidosis type 4)
270.2 Oculocutaneous albinism
243 Pendred syndrome
756.89 Pyle disease
356.3 Refsum disease
Richards-Rundle syndrome
356.2 Sensory radicular neuropathy
Usher syndrome, types 1-3
Wolfram syndrome
Thought to involve Autosomal recessive inheritance
759.89 Mohr syndrome (oral-facial-digital syndrome type 2)
333.2 Myocolonic epilepsy
Male-lethal X-linked inheritance
759.89 Oral-Facial-Digital syndrome
Mitochondrial inheritance
Myoclonic epilepsy with ragged-red fibers (MERRF)
Sporadic occurence
758 Down Syndrome
758.1 Trisomy 13
758.2 Trisomy 18
758.6 Turner Syndrome
X-linked dominant inheritance
759.89 Fronto-metaphyseal dysplasia
759.89 Otopalatodigital syndrome (aka Taybi oto-palato-digital syndrome)
X-linked recessive inheritance
277.5 Hunter syndrome, aka Mucopolysaccharidosis type II
Mohr-Tranebjaerg Syndrome (deafness-dystonia-optic neuronopathy syndrome)
743.8 Norrie disease
X-linked nonsyndromic hearing loss, DFNX
Genetic heterogeneity: Autosomal dominant, Autosomal recessive
757.3 Marshall syndrome
359.1 Muscular dystrophy, including Myotonic Muscular Dystrophy Type 1
756.51 Osteogenesis imperfecta
Genetic heterogeneity : Autosomal dominant, Sporadic
756 Goldenhar syndrome
Genetic heterogeneity: Autosomal dominant, X-linked recessive
759.89 Cornelia de Lange Syndrome
Genetic heterogeneity: Autosomal dominant, Autosomal recessive, X-linked recessive
759.89 Alport syndrome
756.52 Osteopetrosis
Genetic heterogeneity: Autosomal dominant, Autosomal recessive, Sporadic
756.16 Klippel-Feil sydnrome
Genetic heterogeneity: Autosomal recessive, Sporadic
756.0 Pierre Robin sequence
Congenitally acquired causes
343 Cerebral Palsy
771.1 Congenital cytomegalovirus infection
760.71 Fetal alcohol syndrome
Fetal iodine deficiency effects
Fetal methyl mercury effects
Infectious causes
381.00 Otitis media with effusion, chronic
055.9 Measles
Meningitis (viral or bacterial)
072.9 Mumps
034.1 Scarlet fever
760.2 TORCH infections (Toxoplasmosis, Other, Rubella, Cytomegalovirus, Herpes simplex virus type 2; Other can include cosxsackieviurs, syphilis, varicella-zoster virus, HIV, parvovirus B19)
Traumatic causes
872.61 Acoustic trauma, such as from explosions, fireworks, gunfire, rock concerts, and earphones
872.61 Barotrauma (differences in pressure)
801 Skull fracture (temporal bone)
872.61 Traumatic perforation of the eardrum
Toxic causes
E935.5 Aspirin and other salicylates
E930.8 Aminoglycoside antibiotics
E933.1 Cisplatin
E933.1 Carboplatin
E933.1 Carboplatin
E933.1 Dichloromethotrexate (DCM)
E947.8 Diflouromethylornithine (DFMO)
E930.3 Erythromycin
E944.4 Ethacrynic acid
E944.4 Loop diuretics
E947.8 Misonidazole
995.29 Nitrogen mustard
995.29 NSAID
E931.3 Quinine and quinine derivatives
E930.8 Vancomycin
E933.1 Vincristine
Other causes
225.1 Acoustic neuroma
386.00 Meniere disease
Complications of very early prematurity/low birth weight
384.2 Persistent perforation of ear drum

Authors & Reviewers

Initial Publication: August 2013; Last Update: August 2013
Current Authors and Reviewers (click on name for bio):
Reviewer: John C. Carey, MD
Authoring history
(Limited detail is available on authoring dates before 2014.)
AAuthor; CAContributing Author; SASenior Author; RReviewer

Page Bibliography

ICD-9-CM Coordination and Maintenance Committee.
AMA Physician ICD-9-CM 2013, Volumes 1 & 2.
Chicago: American Medical Association; 2013.

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