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.

Timeline

November: Research of types of hearing loss. Genetics involved in hearing loss development and being born with hearing loss. Research how the different types of hearing loss have different impacts in a person's life and what technologies are currently available to help with this.

December / January: research how acoustics can play a role in learning within the classroom. Get in touch with acoustics professional at RPI.

February: Present findings and try to develop a plausible way to achieve a goal of getting the most out of class time with tips and techniques that coincide with my research.

March / April: Using all the research and data collected thus far, I plan to bring that knowledge to the real world and shadow a doctor who specializes in hearing and ears. With these experiences I then will create a presentation that accurately represents my findings of the year and that explains everything that occurred and what I have learned.

May: Finalize project; presentation and edits.