Delay and failure to diagnose cholesteatoma

Presentation

On April 9, 2020, a seven-year-old boy was brought by his mother to see an otolaryngologist (ENT A) on referral from the boy’s pediatrician (Pediatrician A).
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The patient had a history of frequent ear and throat infections with fluid in his ears, nasal congestion, runny nose, coughing, and occasional snoring. The patient had received speech therapy services up until he was three years old for speech delay.
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The patient had undergone an audiogram that showed right conductive hearing loss with fluid in the right ear. The mother was concerned that the ear infections were affecting his ability to hear and contributing to his speech delay.

Physician action

ENT A examined the patient and noted fluid in the left ear and tonsils at 2.5+. He recommended tonsillectomy and bilateral myringotomy with tympanostomy. Surgery was scheduled and informed consent was obtained from the patient’s mother.
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On May 17, ENT A performed the procedures on the patient. During the procedures, ENT A found significant adenoid hypertrophy causing near full obstruction of the nasopharynx. He also performed an adenoidectomy on the patient. No complications were noted. Through the rest of the month, ENT A followed the patient and documented no ear drainage was present, ear tubes remained intact, and the tonsillar bed was healing.
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At a follow-up appointment on September 29, the patient’s mother reported that his speech was improving. ENT A noted that the patient’s ear tubes were visible and open and there was no recurrent ear infection.
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At two follow-up appointments over the next eight months, the patient’s ear tubes were noted as open and patent with mild severity. An audiology test revealed conductive hearing loss in the right ear and an abnormal eardrum. The patient’s mother also reported she was taking her son to a speech therapist.
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Hearing tests conducted over the next four months were consistent with initial findings. On April 20, 2021, the patient’s mother reported her son’s speech development had plateaued. 
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ENT A diagnosed speech and language delay due to hearing loss, bilateral chronic serous otitis media, allergic rhinitis, and conductive hearing loss of the right ear. ENT A prescribed ciprofloxacin and dexamethasone drops for the right ear.
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Follow up audiology testing revealed a Type-B tympanogram of the right ear; the audiologist documented that the right tube was not moving properly. Tests showed conductive hearing loss in the right ear.
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ENT A referred the patient to ENT B for a second opinion. On August 12, 2021, ENT B found the right ear to be very far anterior and inaccessible for patency. ENT B discussed with the patient’s mother either having the patient’s ear tube removed or ordering imaging studies to rule out any tumors or cysts.
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The mother chose to move forward with imaging studies, but there was no documentation that these orders were submitted or there was any follow up from ENT B’s office or the patient’s mother on the studies. 
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Approximately one month later, the patient was seen by ENT C on referral from Pediatrician A for continued hearing loss after tube placement. ENT C noted right ear effusion without acute infection; no tenderness or swelling of the mastoid; and that the patient could detect voices at a conversational level. Her assessment included a clogged right tympanostomy tube. The patient’s mother planned to follow up with ENT A for a possible right tube replacement.
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On September 22, ENT A removed the right ear tube from the right anterior inferior ear drum. Five days later, the patient had a hearing test that showed continued hearing loss in the right ear.  A tympanogram showed an abnormal right eardrum.
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On referral from Pediatrician A, the patient was taken to see ENT D on November 21 for hearing loss. ENT D ordered a CT of the temporal bone to rule out a middle ear mass. A CT performed on December 10 showed a right-sided cholesteatoma with partial erosion of the ossicular chain and partial dehiscence of the tegmen tympani. ENT D advised that surgery was required.
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On February 7, ENT E performed a right tympanoplasty and mastoidectomy and right posterior auricle split-thickness skin graft. Reconstruction surgery was performed on November 12. Recurrent cholesteatoma was found in the sinus tympani and a small external canal cholesteatoma in the anterior sulcus.
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A hearing test conducted a year later found the patient’s hearing remained essentially stable with a slight decrease in the right ear. 

Allegations

The patient’s family filed a lawsuit against ENT A with allegations of delay and failure to diagnose cholesteatoma resulting in permanent hearing loss.

Legal implications

Expert consultants for both the plaintiff and defense agreed that congenital cholesteatoma is rare, difficult to diagnose, and that a diagnosis is often delayed without persistent conductive hearing loss or a visible mass in the middle ear behind the eardrum. The condition is more likely to develop slowly in adults. The patient was seen by several ENT physicians without a diagnosis of cholesteatoma. 
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One defense expert stated that the cholesteatoma would not have been seen by the various ENTs treating the patient because of its location. The cholesteatoma was in the posterior aspect of the middle ear near the ossicles. The expert explained that when one looks in the ear with a scope, the anterior aspect is the only visible part of the ear. This expert added that a CT immediately after myringotomy would also not have found the cholesteatoma due to its location and size (being too small and not visible).
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An otolaryngology consultant for the defense believed that the patient’s hearing loss was caused by ossicular chain damage from cholesteatoma that had occurred before the patient was seen by ENT A. This consultant felt it was not reasonable to expect ENT A to suspect cholesteatoma in a young child. Instead, given the patient’s symptoms, age, and number of ear infections, it was reasonable and “within the standard of care” for ENT A to remove the tonsils and place ear tubes.
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This consultant did point out that there was a delay in obtaining imaging studies to rule out tumors or cysts. There was no documentation by ENT A or ENT B existed that these tests were ordered. While overall care provided to the patient was appropriate, earlier imaging may have identified the cholesteatoma sooner.
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An expert consultant for the plaintiff could not make a causal link from ENT A’s care of the patient to specific damages. He could only testify that “an earlier diagnosis would have been better.”

Disposition

This case was taken to trial, and the jury returned a verdict in favor of the plaintiffs.

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Risk management considerations

In this case, the patient’s young age may have been a factor in the diagnosis delay — and the sympathies of the jury. When treating minors, the normal physiological and developmental changes that occur in the patient over time may contribute to a delayed or missed diagnosis, especially in the event of a rare condition such as congenital cholesteatoma.
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ENT A and B were both criticized for not obtaining imaging studies that may have identified the cholesteatoma in a timelier manner. When recommending and agreeing to diagnostic tests for a patient, it is the physician’s responsibility to order the test; document the order; obtain the test results in a timely manner (if the patient is compliant); review the results when received; document his or her review in the medical record; and initiate appropriate follow up with the patient.
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Had imaging studies been obtained earlier in this case, the patient’s cholesteatoma may have been diagnosed earlier. It was unclear why the tests discussed with the mother were not ordered. If a patient or parent decides not to proceed with a recommended test, their decision should be documented in the medical record.
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To avoid risks associated with a failure to follow up, physicians may consider establishing clear policies and procedures for patient follow up. Adopt technologies in the electronic health record (EHR) system that employ built-in systems such as reminders for testing or alerts when results of ordered testing have not been received. Consider prioritizing test results with “urgent,” “critical,” “action needed,” or “pending results.”
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There was also concern that ENT A made documentation errors in the patient record, such as mistaking whether the patient’s right or left ear was affected. There were also patient visit notes that were added to the record up to a week after the patient was seen.
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Clear, contemporaneous documentation helps to ensure that critical data is available to all treating health care providers and helps to maintain continuity of care. Together, timely consults, testing, and comprehensive medical records, that are accessible by the entire care team, can help catch potential diagnostic errors or oversights before they negatively affect patient outcomes.