Understanding Congenital Sensorineural Hearing Loss

What is sensorineural hearing loss? 

Sensorineural hearing loss (SNHL) happens when there is damage or malformities to tiny hair cells in the cochlear and/or the auditory nerve. In children, the most common causes of SNHL include inner ear abnormalities, genetic variations, jaundice, and viral infection from the mother during pregnancy. In adults, SNHL is most commonly caused by aging, exposure to loud noises, head trauma, or other conditions. 

My diagnosis story 

I was born just six hours too early to receive the piloted Welsh NHS newborn hearing test in December 2003! Because of this, I was first diagnosed with this condition when I was five years old, in 2009. I was just entering year one of primary school. Concerns about my hearing were first raised in my pre-school general assessment when I couldn’t hear the assessor when she was behind me to the right. As a result, I was referred to an audiologist for further testing and was diagnosed with moderate/severe mid to high frequency unilateral (right side) congenital sensorineural hearing loss. My left ear hears in a normal range. By all professional estimations, it’s likely I was born with this hearing loss. When I was 10, I had to have an MRI scan to try to determine the cause of this hearing loss. No physical abnormalities were found, so the problem lies at a cellular level in the nerves of the cochlear. 

Having this condition means I struggle hearing across a distance, I struggle to localise where a sound is coming from, and I struggle to focus my listening when there is background noise. Clarity of speech is a big issue. Due to the abnormalities in my auditory nerves, I also experience a paradox where I am hard of hearing but also extremely sensitive to loud noises and busy environments, such as in a bar, concert, busy station, etc., and I actively seek to avoid these environments where possible. I also struggle to differentiate between spoken consonants, which translates into my writing and speech as the sounds are the same, affecting my perception of words. Learning languages is near impossible. 

Since diagnosis, I have had access to a hearing aid for my right ear. I usually just went without as I managed fine in school, but now part of my daily ritual is ensuring I am wearing one. My first hearing aid had a clear mould with a tiny Super Mario figure inside of the mould - I was the coolest kid in Barry Town! The hearing aid I now wear is quite a glamorous?pink aid with a pink-glitter mould, but I still miss my Mario one… 

My experience in education 

During school, my hearing loss wasn’t a massive concern. Adjustments were made in the classroom such as seating plans at the front, repeated instructions, and one-to-one as needed. My biggest issue has been since starting university. I often struggle to hear lecturers across a busy, echo-ey lecture hall, so I make an effort to sit near the front. I also struggle with different accents to my own, which isn’t an issue in a small school in South Wales, but has become a big problem at uni.? 

Since I qualified for DSA in January 2023, I now have access to a Phonak Roger microphone, which connects over a secure radio frequency directly into my hearing aid, enabling me to hear lecturers more clearly. It’s like someone has stopped muffling the speaker! 

Throughout all my life in education, when I have had to ask people to repeat themselves, I have often experienced “are you deaf” as a hurtful line of questioning. I rely heavily on lip reading, which used to be an issue in classroom-type settings but is far easier in lectures now. 

Misconceptions 

Being deaf or hard of hearing is a diverse range of conditions. I consider myself hearing and do not identify with the term disabled. Others with the same condition may feel the opposite about this terminology. Most people are not 100% either deaf of hearing. I often find myself on the receiving end of “you’re young, you’ve got good ears” assumptions, which is definitely not the case. My hearing loss is usually an acquired condition and isn’t usually observed in children, so I get this all too often. 

Advice 

Take steps to protect your hearing. Avoid extremely noisy environments where possible. If your hearing is poor or “isn’t what it used to be”, take a test with a professional audiologist. 

Be considerate of those around you. If someone keeps asking you to repeat yourself, it isn’t because they’re ignorant, it may be because they can’t hear you! 

There are ongoing debates within the Deaf communities regarding the use of the term “hearing impaired” vs “hard of hearing”. While I personally prefer hearing impaired, others may prefer hard of hearing so it’s always best to ask.