Q.M. is also a patient who received services on the NSU audiology mission to a Caribbean island.  The audiology technician for the island had conducted OAE testing on Q.M. repeatedly over the years.  The right ear has had absent OAEs and the left ear, present.  They were confirmed as present in the left ear, and possibly present in the high frequencies in the right ear.

His mother has neurofibromatosis, and so does QM.  It was not noted whether it was type 1 or 2 neurofibromatosis.  His mother denies hearing loss, but her speech sounds hearing impaired.  The impression she gave on initial meeting was that she may have limited cognitive ability herself; however, on the second visit, after longer interactions, this seemed less certain.

When he was seen, his right ear had a flat tympanogram, and his left ear had normal results.

The 5 year, 10 month old was extremely challenging to test, as shown by the “scratch” audiogram.

Because of the uncertainty about the results, he received ABR testing.  There was history of previous failure to sedate for imaging testing.

Prior to the team’s arrival, and by our request, the sedation (chloral hydrate, by choice of the physicians on the island) was to be administered by the physician and the patient was to be tested in the pediatric ward of the hospital.  The head pediatrician questioned the necessity of that arrangement “for just chloral hydrate”, preferring not to have the activity in the pediatric ward, instead preferring that the testing be conducted in the area of the hospital where the other audiology testing was conducted (outpatient services area).  The audiology professor expressed unwillingness to conduct testing without medical monitoring.  A compromise was reached, for that day testing would be in the pediatric hospital ward; subsequent days an EMT with resuscitation equipment would be present in the audiology area.

QM was given oral chloral hydrate by a physician.  The child became hyperactive. It is possible that a second dose was given; the audiology team was not monitoring the patient.

When the patient was sedated, click ABR testing was conducted.  Interpeak latencies were prolonged bilaterally – 4.88 ms in the right ear and 4.55 ms left.

• For a child approaching age 6, how delayed are those latencies?
• Given the unknown type of neurofibromatosis, how is this interpreted?

Threshold testing was attempted; a binaural response at 60/70 at 500 Hz was obtained, and 3k Hz tone burst thresholds were 60 dB nHL left,and 40 dB nHL right.  Q.M. alternated between snoring, and having short episodes of apnea.

• Does this substantiate the behavioral testing?

Unfortunately, testing was unable to continue.  The child was having increasingly long and severe episodes of apnea.  The audiology faculty member sat on the child’s bed, frequently repositioned the child and tried to arose him with shaking, which was unsuccessful. Mildly noxious tactile stimulation, such as rubbing knuckles on the breast bone, failed to awaken the child.

Chloral hydrate is presumed to cause “minimal sedation.”

• Is this patient’s response typical of minimal sedation?

The audiologist’s repositioning of the child kept the apnea episodes to around 15 seconds each, but they were increasingly successive.  While the medical team was being summonded, the child voided; the audiologist wished she had not been sitting on the bed.   He was placed in the care of the medical team and remained hospitalized overnight.

Although an ear impression was taken, the team was hesitant to recommend amplification.

• Discuss the probably reasons for reluctance for trial amplification.

ENT referral had been obtained.  On the return visit, repeat behavioral testing was prioritized.  The child again was extremely hyperactive, with behavior more typical of a 3 year old than a child now 6.

Shown below is audiologist Dianne Cooper, AuD, trying to coax responses from QM.  If you look carefully, you can see that he is seated on the lap of another audiologist, who has one leg wrapped around the child’s waist to restrain him.  Without that intervention, QM would not remain seated.  Testing was even less successful than what was obtained on the first visit; mild unilateral loss appeared to remain inspite of the medical intervention.

 

The child’s mother very much wanted amplification for QM.  In consultation with the audiology technician who arranged for the mission, the decision was made that the child would try amplification when receiving individualized speech therapy services.  The aid would not be used in the classroom or at home at this point; the audiology technician has the ability to monitor the situation and alter the decision.

• Typically, it is the parent who makes the decision for the child.  Did the audiology team overstep by requiring that the donated aid be used during therapy only?

The mother reported that QM is on a CPAP machine for his sleep apnea.  She pointed out QM’s neck tumor, a consequence of his NF.  His normal side of the neck and tumor side are shown; picture quality is poor.  (One of the children had played with the camera and changed the setting away from automatic which wasn’t realized at the time.)

• Does the presence of a tumor suggest it is type I or type II neurofibromatosis?