Clinic - Week # 6

This week in clinic I didn't seen anything exciting, so I deiced to research a article and post something on a topic we don't see everyday.

I looked up Goldenhars syndrome. This is a rare, sporadic syndrome that was founded in the 1950's. Characteristics of this syndrome malformation of the ear, auricular appendices, vertebral anomalies, occulomotor problems, hemifacial microsomia, and agenesis and hypolasia of the mandible. Some other characteristics could be cleft lip and palate, central nervous system, and visceral anomalies.

Diagnosis is hard because not enough information is readily available from previous cases. Some chromosomes have been identified and a suggested disturbance of neural crest cells could be the cause of the disease. Environmental factors such as drugs and maternal diabetes have also been suggested as causes of the disease.

Clinical relationship: Patients with Goldenhars could have microtia and appendices of the ear, mandibular hypoplasia, palpebral alteration, lateral facial clefts (look for a conductive hearing loss associated with clefting problems), and renal problems.

Here is a case study of an 11 year-old, white, female with Goldenhars. She presented with facial asymmetry, hypoplasia of the mandible, dermoid epibulbar tumor on the left eye, and birthmarks on the upper lip and palate. No mental problems were detected during testing. The mother reported used of an anti-convulsive drug (Comital) because of epilepsy prior to knowing of the pregnancy. After, the drug was changed to Phenobarbital Gardenal which is more suitable for use during pregnancy. Dental development was normal, and an x-ray of the skull and vertebral column did not show any abnormalities. Hypoplasia of the mandible was seen on the left side and absence of the coronoid process was observed.

The case study mentioned that this condition is controversial because of the complexity and the broad clinical aspects.

Check it out!!

http://www.scielo.br/pdf/bdj/v14n1/v14n1a13.pdf

Clinic Week # 5

This week in clinic I saw an adult male who came in for his hearing aid orientation. Twenty years ago he wore an ear level tinnitis masker and wanted something similar that would help mask his high frequency tinnitis. A few weeks ago he was seen in our clinic for a hearing evaluation and at that time decided to purchase hearing aids. He purchased bilateral Oticon Delta's. Today his Oticon Delta's were programmed using NOAH and Genie. His left hearing aid was reprogrammed after he said that is sounded too loud. He was instructed on the use and care and his new hearing aids and how to change the batteries. He was reminded of the 30-day trial period and instructed to return to our clinic before that date if he wanted to return the hearing aids.

The Client Orientated Scale of Improvement (COSI) was administers and his specific need was to help mask his tinnitis. The COSI will be readministered when the patient returns. The QuickSIN speech-in-noise test was also administered and his mean score improved from 12.8 to 4.5 signal-to-noise loss. This suggests that the hearing aids were helping him understand speech, especially high frequency sounds.

Later that day, the patient returned to our clinic because he was describing his right hearing aid as "hissing" and "sounding like water on the ground." He thought his wife had left her car door open. The gain was decreased for soft sounds at 1.5 k Hz and the Maximum Power Output (MPO) was increased by 3 dB. After these adjustments the client said the sound had stopped.

This patient brought in articles to share with us on stem cell research and how they are trying to find a way to re-grow the hair cells in the inner ear and in effort to help those with significant hearing loss. I thought this was a neat topic and wanted to tie it in with this patient as my journal link. This article is from the Hearing Exchange. The University of Michigan has a 22 million dollar grant that will fund 5 top researchers, each in the amount of $200,000 for a three year study. In order to make the cells grow again, the researchers need to a successful method to plant and grow the stem cells in the inner ear. They hope to accomplish this in animals, and then later in humans. Recently they used gene techniques to regenerate hair cells in guinea pigs. The hair cells were missing but other supporting cells remained.

I'm listing a few website on this topic. Check them out, it's pretty cool stuff !!! Just think if they could find a way to actually regenerate the hair cells in the inner ear !!!!

http://www.hearingexchange.com/blog/?p=68

http://www.sciencedaily.com/releases/2007/04/070405170200.htm