ETIOLOGY

Most CHL is acquired, with middle-ear fluid the most common cause.

Congenital causes include anomalies of the pinna, external ear canal,

TM, and ossicles. Rarely, congenital cholesteatoma or other masses in

the middle ear manifest as CHL. TM perforation (e.g., trauma, OM),

ossicular discontinuity (e.g., infection, cholesteatoma, trauma), tympanosclerosis,

acquired cholesteatoma, or masses in the ear canal or

middle ear (Langerhans cell histiocytosis, salivary gland tumors,

glomus tumors, rhabdomyosarcoma) also can manifest as CHL.

Uncommon diseases that affect the middle ear and temporal bone and

can manifest with CHL include otosclerosis, osteopetrosis, fibrous dysplasia,

and osteogenesis imperfecta.

SNHL may be congenital or acquired. Acquired SNHL may be

caused by genetic, infectious, autoimmune, anatomic, traumatic, ototoxic,

and idiopathic factors (Tables 637-1, 637-2, 637-3, and 637-4).

The recognized risk factors account for approximately 50% of cases of

moderate to profound SNHL.

Sudden SNHL in a previously healthy child is uncommon but may

be from OM or other middle-ear pathologies such as autoimmunity.

Usually these causes are obvious from the history and physical examination.

Sudden loss of hearing in the absence of obvious causes often

is the result of a vascular event affecting the cochlear apparatus or

nerve, such as embolism or thrombosis (secondary to prothrombotic

conditions), or an autoimmune process. Additional causes include

perilymph fistula, drugs, trauma, and the first episode of Meniere

syndrome. In adults, sudden SNHL is often idiopathic and unilateral;

Neonates (Birth-28 Days) When Universal Screening Is Not Available

Family history of hereditary childhood sensorineural hearing loss

In utero infection, such as cytomegalovirus, rubella, syphilis, herpes simplex, or toxoplasmosis

Craniofacial anomalies, including those with morphologic abnormalities of the pinna, ear canal,

ear tags, ear pits, and temporal bone

anomalies

Birthweight <1500 g (3.3 lb)

Hyperbilirubinemia at a serum level requiring exchange transfusion

Ototoxic medications, including but not limited to the aminoglycosides, used in multiple courses

or in combination with loop diuretics

Bacterial meningitis

Apgar scores of 0-4 at 1 min or 0-6 at 5 min

Mechanical ventilation lasting ≥5 days; extracorporeal membrane oxygenation

Stigmata or other findings associated with a syndrome known to include a sensorineural and/or

conductive hearing loss;

Infants and Toddlers (Age 29 Days-2 Yr) When Certain Health Conditions Develop That

Require Rescreening
Parent or caregiver concern regarding hearing, speech, language, and/or developmental delay

Bacterial meningitis and other infections associated with sensorineural hearing loss

Head trauma associated with loss of consciousness or skull fracture

Stigmata or other findings associated with a syndrome known to include a sensorineural and/or

conductive hearing loss; neurofibromatosis,

osteopetrosis, and Usher Hunter, Waardenburg, Alport, Pendred, or Jervell and Lange-Nielsen

syndrome

Ototoxic medications, including but not limited to chemotherapeutic agents or aminoglycosides

used in multiple courses or in combination

with loop diuretics

Recurrent or persistent otitis media with effusion for 3 mo or longer

Skeletal dysplasia

Infants and Toddlers (Age 29 Days-3 Yr) Who Require Periodic Monitoring of Hearing

Some newborns and infants pass initial hearing screening but require periodic monitoring of

hearing to detect delayed-onset sensorineural

and/or conductive hearing loss. Infants with these indicators require hearing evaluation at least

every 6 mo until age 3 yr, and at appropriate

intervals thereafter

INDICATORS ASSOCIATED WITH DELAYED-ONSET SENSORINEURAL HEARING

LOSS

Family history of hereditary childhood hearing loss

In utero infection, such as cytomegalovirus, rubella, syphilis, herpes simplex, or toxoplasmosis

Neurofibromatosis type 2 and neurodegenerative disorders

Cogan syndrome (vasculitis: keratitis, uveitis, vertigo, arthritis, dermatitis)

INDICATORS ASSOCIATED WITH CONDUCTIVE HEARING LOSS

Recurrent or persistent otitis media with effusion

Anatomic deformities and other disorders that affect eustachian tube function

Neurodegenerative disorders