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Delayed onset hearing loss in a two-year old
Insights
This case study documents a rare instance of delayed onset sensorineural hearing loss in an infant. Surgical repair of bilateral fistulas may have halted and potentially reversed the hearing degradation.
Area of Science:
- Otolaryngology
- Genetics
- Pediatric Audiology
Background:
- Delayed onset sensorineural hearing loss (DSNHL) is a significant concern in pediatric audiology.
- While often hereditary, the underlying causes can be complex and multifactorial.
- Early identification and intervention are crucial for managing hearing loss in infants.
Observation:
- A previously healthy infant presented with normal hearing until 13 months of age.
- A mild high-frequency hearing impairment was incidentally detected at 25 months.
- The hearing loss rapidly progressed to severe levels within six months.
Findings:
- This represents the youngest documented case of hereditary delayed onset sensorineural hearing loss.
- The patient had bilateral oval and round window fistulas, a previously unrecognised factor.
- Surgical repair of the fistulas was performed.
Implications:
- Surgical intervention for congenital fistulas may be a critical factor in managing certain types of DSNHL.
- This case highlights the importance of thorough audiological and anatomical evaluation in cases of rapidly progressing hearing loss.
- Further research is warranted to explore the link between window fistulas and delayed onset hearing loss.
Abstract:
This paper describes a delayed onset sensorineural hearing loss in a baby known to have normal hearing until at least 13 months. A mild high-frequency loss was discovered quite by accident at 25 months. The bilaterally symmetrical loss progressed over the next 6 months to no measurable high-frequency hearing and a 70 dB threshold at 500 Hz. To our knowledge this is the youngest completely documented case of what has been called hereditary delayed onset hearing loss. However, in this instance, and perhaps unsuspected in others, this child had bilateral oval and round window fistulas whose repair may have not only arrested the progression of her loss, but even improved her residual hearing sensitivity.