Tuesday, November 29, 2016

Hearing loss is known for having a genetic component. Two specific genes known for being related to loss of hearing are the GJB2 and GJB6 genes although mutations in at least 30 genes have been identified. The scientific name for the most common form of hearing loss is nonsyndromic hearing loss. Nonsyndromic hearing loss means that there are no other symptoms or signs associated with the hearing loss. Nonsyndromic hearing loss can be inherited in an autosomal dominant pattern; the mutation that causes the hearing loss is on a dominant non-sex chromosome so a person only needs one mutated gene to be affected by the alteration.

Hearing is measured in decibels (dB). The threshold or 0 dB mark for each frequency refers to the level at which normal young adults perceive a tone burst 50% of the time. Hearing is considered normal if an individual's thresholds are within 15 dB of normal thresholds. The chart down below demonstrations the severity of the hearing loss by showing how many decibels away from the threshold the hearing loss is.

Severity of Hearing Loss in Decibels (dB)
Severity
Hearing Threshold in Decibels
Mild
26-40 dB
Moderate
41-55 dB
Moderately severe
56-70 dB
Severe
71-90 dB
Profound
90 dB



The type of hearing loss that I have been diagnosed with is called familial nonsyndromal Mondini dysplasia. Mondini dysplasia is a type of inner ear malformation that is present at birth. Individuals with Mondini dysplasia have one and a half coils of the cochlea instead of the normal two and a half coils. The cochlea receives sound in the form of vibrations, which cause the stereocilia(tiny hairs inside the cochlea) to move. The stereocilia then convert these vibrations into nerve impulses, which are taken up to the brain to be interpreted. With less turns of the cochlea, there are less stereocilia to send signals to the brain about sound, therefore causing hearling loss. Mondini dysplasia may occur in one ear (unilateral) or both ears (bilateral) and can cause varying degrees of sensorineural hearing loss, although most individuals have profound hearing loss. My particular case of Mondini dysplasia is bilateral however I have mild to moderate hearing loss, not profound hearing loss like the majority of those with this abnormality.

1 comment:

  1. Great post! You include lots of interesting fact, as well as some compelling personal information.

    Please include some references for information that is not your own, like the data chart.

    Keep up the great posts!

    ReplyDelete