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 ICD-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).
Several of the ICD-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 |