Fluctuating and Progressive Hearing Loss
Fluctuating and progressive hearing loss can be seen in both syndromic and nonsyndromic forms of hearing impairment. It has been suggested that a change in auditory
threshold of 10 dB at any test frequency be used to define a significant change in hearing sensitivity with smaller changes
being within the range of normal test-retest variability. It is estimated that between 2 and 33% of children with sensorineural
hearing loss show progressive loss of auditory sensitivity. Some forms of hearing loss associated with a fluctuant and/or
progressive coarse are listed below.
Genetic Syndromes
- Alber-Shonberg disease
- Alport syndrome
- Branchio-otorenal syndrome
- CHARGE association
- Cockayne syndrome
- Friedreich ataxia
- Mucopolysaccharidoses (Hurler, Hunter, and Marateaux-Lamy syndromes)
- Neurofibromatosis
- Norrie syndrome
- Osteogenesis imperfecta
- Osteopetrosis
- Pendred syndrome
- Refsum syndrome
- Stickler syndrome
- Waardenburg syndrome
- Congenital cytomegalovirus
- Congenital rubella
- Congenital syphilis
- Herpes simplex virus
- Varicella
- Measles
- Hyperbilirubinemia
- Persistent pul
- Mondini dysplasia
- Sheibe dysplasia
- Large vestibular aqueduct syndrome
- Hyperbilirubinemia
- Persistent pulmonary hypertension of the newborn
- Autoimmune inner ear disease
- Meniere's disease
- Meningitis
- Perilymphatic fistula
Obtaining frequent audiograms in children with hearing loss may help to identify
progressive or fluctuating forms of hearing loss and can help to ensure the adequacy
of treatment. Children with newly identified sensorineural hearing loss should
have audiograms every 3 months during the first year, every 6 months during their
preschool years, and at least once a year while in school. Children identified
with a fluctuating or progressive hearing loss should be seen by a pediatric otolaryngologist
as soon as possible. Early medical intervention may reverse or stabilize this
process.
[Bluestone: 1996]
[Bluestone: 1996]
Page Bibliography
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Pediatric Otolaryngology.
Philadelphia: Saunders College Pub;
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Tharpe AM, Bess FH.
Minimal, progressive, and fluctuating hearing losses in children. Characteristics, identification, and management.
Pediatr Clin North Am.
1999;46(1):65-78.
PubMed abstract
Tomaski SM, Grundfast KM.
A stepwise approach to the diagnosis and treatment of hereditary hearing loss.
Pediatr Clin North Am.
1999;46(1):35-48.
PubMed abstract