Background Information: AG is a 35-year-old female who was seen in ENT clinic. She presents with Osteogenesis Imperfecta (OI). Due to this disorder, she underwent a stapedectomy in the right ear when she was 16 years old. She reports that she has had a change in hearing of her right ear; it is suspected that the prosthesis in right ear has slipped.  At the time that she was seen, a CT scan had been ordered.

Osteogenesis Imperfecta: Osteogenesis Imperfecta is also known as “brittle bone disease.” It is due to a mutation of several genes that are responsible for processing collagen, which is crucial for bone strength and toughness. Common associated features of OI include a short stature, blue sclerae on the eye, and fractures following minor injury. Collagen in the ear involves all auditory structures and due to the deficient production, often leads to early onset hearing loss. As those with OI continue to age, ossicular issues become very common.

Common audiologic management includes the placement of pressure equalization (PE) tubes to manage recurrent ear infections. Stapedectomies, like AG had, are common as well when there are ossicular issues. Since individuals with OI have weaker supporting bones, it is easier for stapes footplate fixation or even fractures of the ossicles. A stapedectomy would help tackle this issue, however; outcomes for those with OI who get this procedure have a higher likelihood of a failure of the prosthesis. Bone-anchored-hearing-aids (BAHA) are also a common hearing device for those with OI, as conductive hearing loss is often seen in those with OI due to middle ear issues.

Audiologic Information: Due to the hectic nature of ENT clinic and the likelihood of a slipped prosthesis in the right ear, tympanograms were not performed bilaterally. Audiometric results revealed a profound, rising to moderately-severe conductive hearing loss in the right ear and normal hearing acuity in the left ear.

At a previous appointment three months earlier, word recognition testing was performed. Word recognition was tested utilizing the recorded NU-6-word list. Scores were 96% with a presentation level of 90 dB HL (40M) in the right ear and 100% at 60 dB HL in the left ear.

Discussion: Based on the results of today’s testing, it is recommended that AG pursue amplification for the right ear. It is likely that the resulting hearing loss is due to a slipped stapes prosthesis, but scans are needed to confirm this.

The ENT discussed amplification options, including but not limited to a Bone Anchored Hearing Aid (BAHA), power hearing aid for the left ear, or a CROS hearing aid.

As of this date, a hearing aid evaluation has been scheduled. So, we shall see the route that AG chooses! Stay tuned….

What would you do?


Pillion, Joseph P., David Vernick, and Jay Shapiro. “Hearing Loss in Osteogenesis Imperfecta: Characteristics and Treatment Considerations.” Genetics Research International 2011 (2011): 1-6.

Jessica Minnick is a fourth-year graduate student at the University of Louisville in Kentucky. She is completing her externship at the Cleveland Clinic in Cleveland, Ohio. Her audiological interests include vestibular assessment, hearing aids, and educational audiology.


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