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

Acquired Causes

• Congenital cytomegalovirus
• Congenital rubella
• Congenital syphilis
• Herpes simplex virus
• Varicella
• Measles
• Hyperbilirubinemia
• Persistent pul

Inner Ear Malformations

• Mondini dysplasia
• Sheibe dysplasia
• Large vestibular aqueduct syndrome

Other

• Hyperbilirubinemia
• Persistent pulmonary hypertension of the newborn
• Autoimmune inner ear disease
• Meniere's disease
• Meningitis
• Perilymphatic fistula

Identifying fluctuating or progressive hearing loss may be difficult, particularly during early childhood. Sometimes changes in behavior or declines in academic performance caused by a decrease in hearing sensitivity are misinterpreted by parents and physicians as defiant behavior and limit-testing. Health professionals may also incorrectly assume that changes in audiometric results are due to attentional or behavioral factors, as opposed to significant changes in auditory sensitivity. Physicians must maintain a high index of suspicion for fluctuating or progressive hearing loss and follow through with prompt audiologic evaluation, identification of causative factor(s), and appropriate intervention.

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]

[Tharpe: 1999]

[Tomaski: 1999]