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UAE: Doctors perform groundbreaking surgery to restore patient's hearing

Shaikh Shamshuddin was suffering from a genetic condition that resulted in near total hearing loss

Doctors in Abu Dhabi have performed a ground-breaking auditory brainstem implant (ABI) on a patient suffering from a genetic disease that led to multiple large benign intracranial tumours resulting in near total hearing loss.

The implant was the available option for the patient to recover some hearing and not go completely deaf.

Shaikh Shamshuddin, a 36-year-old male, had neurofibromatosis – a genetic condition that had, over time, led to the growth of several benign brain tumours. He underwent surgery at another hospital that was unable to remove all of his tumours. His condition worsened, and he developed two large tumours in his brain that were compressing crucial areas that impacted his hearing. By the time Shamshuddin was admitted to Cleveland Clinic Abu Dhabi, he was experiencing nearly complete hearing loss and his family had become increasingly concerned about his condition.

“I couldn’t hear through both my ears, and we were praying to get a good doctor and a good hospital,” said Shamshuddin.

Dr Florian Roser, Chair of the Neurological Institute at Cleveland Clinic Abu Dhabi, explained to Shamshuddin and his family that the tumour permanently affected the functioning of the hearing nerve, which meant an external hearing aid wouldn’t solve his problem. Instead, Dr Roser suggested a procedure called an auditory brainstem implant (ABI).

“Shaikh was a patient with a genetic disease leading to multiple large benign intracranial tumours. These were not only potentially life-threatening, but patients with these types of tumours often lose hearing as the tumours develop close to the hearing nerve. The plan was to first take care of the tumour resection as a life-saving procedure. At the same time, we needed to find a solution for his hearing loss. Implantation of an auditory brainstem implant was the best option for him, as it would help him regain some of his hearing capacity,” Dr Roser said.

What is ABI?

While not capable of granting a patient entirely new hearing, an ABI can still help them detect sounds. It is a surgically implanted device that provides a sensation of sound to a person who has severe hearing loss due to damage to the auditory nerve. Although the design and function of the cochlear and auditory implants are similar, the devices are different. The cochlear implant is used in individuals with cochlear damage, but they still have a functioning auditory nerve. The auditory brainstem implant on the other hand, bypasses the inner ear and the auditory nerve. It uses an array of electrodes to stimulate the hearing pathways on the brain stem directly.

“It’s not as though when we switch the device on, you have new hearing. However, many patients have reported improvements in their sound awareness and lip-reading abilities after an ABI,” Dr Roser pointed out.

Complex procedure

Cleveland Clinic Abu Dhabi now joins an exclusive list of worldwide centres offering ABI surgery.

A UAE-based surgical team performed this procedure on Shamshuddin. This entiled two delicate surgeries to resect the brain tumours first, which were high risk procedures, as crucial centres of the brainstem were involved. The first step involved removing the tumour on the side where the implant would be positioned. The second vital step was the preservation of the facial nerve to retain his facial expression and movement, his ability to smile and close his eyes, and then, the delicate and intricate placement of the implant. As a third step following the tumour surgery, the implant was positioned in a specific sub-millimetre area of the brainstem to receive auditory stimulation right on the brain. This cable was then connected to a computer device implanted behind the ear under the skin.

“Dr Florian took all fear away from me. He explained everything to me as if I was his family,” Shamshuddin said.

Hearing improved

A multidisciplinary team of neurosurgeons and ENT surgeons together successfully completed the surgery on Shamshuddin to the delight of both him and his overjoyed family. After the implant was activated and three months following the surgery, Shamshuddin was able to hear sounds that helped him function in his everyday life, helping him to orientate himself. For example, hearing the fire alarm, direction from where cars are approaching, and the phone ringing.

“I was very excited when the implant was activated for the first time. The beeping sound of the monitor was the first sound I heard after about a month and a half of complete hearing loss. After three months my hearing slowly improved, and I can now understand a little when someone is speaking. I cannot express my gratitude enough to Cleveland Clinic Abu Dhabi for how they have changed my life,” Shamshuddin added.

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