Answers and More Questions

On Monday, we took Riley to the hospital for her Auditory Brainstem Response Test. We were to find out if she had any hearing loss and to what extent she had. She got tubes in her ears in March after having chronic, monthly ear infections since October of last year. We hoped that after getting the tubes her speech would miraculously improve. This was not the case. Yes, she had great improvements but not to the extent that we had hoped for. At a recent followup with the ENT after several failed hearing tests in the sound booth, he decided to conduct the aforementioned test to find out what was happening.

Riley enjoying computer games at the library this summer.

The test only took an hour or so and we were able to get the results right away. While we expected by that point to hear that she had some hearing loss, we figured there was just some frequencies she could not hear, we were not prepared for what they told us. Riley has Unilatoral Sensorineural Hearing Loss in her left ear. See the technical description below. Basically she can not hear anything in that ear and has not for some time.

Luckily, her right ear functions just fine. She can not tell where sound is coming from but she can hear. The good thing with the right ear functioning is that it is tied to the left brain which has speech and language control.

We do not know when the hearing loss occurred, how it occurred or if it is reversible. Over the next few months we will be meeting with a geneticist and she will have a lot of tests runso in a few months we may have some answers.. They will be monitoring her right ear every couple of months to make sure nothing happens to it. At this time we don’t know if a hearing aid will help her or not. The audiologist at the hospital mentioned a BAHA, bone anchored hearing aid that screws into the head, but the don’t do those until a child is older.

This diagnoses answers some major questions. We know now why she is not progressing as she should. It also explains her behavior issues. She does not like noisy settings as she can not localize where the sound is coming from. She also gets frustrated when we can not understand what she is trying to tell us. For now, we make sure she is paying attention to us when we are speaking to her, speak to her in her right ear and help her cope with chaotic, loud situations as best as we can.

We now know what path we are on but have a long way to go until we reach the end of the road. Once we know more we will keep you posted.

Sensorineural Hearing Loss
From Wikipedia, the free encyclopedia

Sensorineural hearing loss is a type of hearing loss in which the root cause lies in the vestibulocochlear nerve (Cranial nerve VIII), the inner ear, or central processing centers of the brain. Sensorineural hearing loss can be mild, moderate, or severe, including total deafness. Riley’s is considered severe/profound.

The great majority of human sensorineural hearing loss is caused by abnormalities in the hair cells of the organ of Corti in the cochlea. There are also very unusual sensorineural hearing impairments that involve the VIIIth cranial nerve (the Vestibulocochlear nerve) or the auditory portions of the brain. In the rarest of these sorts of hearing loss, only the auditory centers of the brain are affected. In this situation, central hearing loss, sounds may be heard at normal thresholds, but the quality of the sound perceived is so poor that speech can not be understood.

Most sensory hearing loss is due to poor hair cell function. The hair cells may be abnormal at birth, or damaged during the lifetime of an individual. There are both external causes of damage, like noise trauma and infection, and intrinsic abnormalities, like deafness genes.

Sensory hearing loss (also called sensorineural hearing loss) may also result from abnormalities of the VIII cranial nerve.

Sensory hearing loss that results from abnormalities of the central auditory system in the brain is called Central Hearing Impairment. Since the auditory pathways cross back and forth on both sides of the brain, deafness from a central cause is unusual.

This type of hearing loss can also be caused by prolonged exposure to very loud noise. For example, wearing headphones on full blast for a few hours.