Symptom: Unilateral Facial Paralysis

What's Your Diagnosis? Symptoms: Middle Ear Mass and Unilateral Hearing Loss

Symptom: Unilateral Hearing Loss

Symptoms: Sudden Sensorineural Hearing Loss and Aural Fullness

Symptom: Postauricular Mass

The patient’s imaging features, especially the honeycomb pattern of ossific changes in the geniculate fossa, were virtually pathognomonic for ossifying hemangioma of the facial nerve.

Symptom: Conductive Hearing Loss after Cholesteatoma Surgery

Symptom: Deafness After Stapedectomy

A 30-year-old man with prior diagnoses of Bell's palsy and migraine headaches presented with fourth months of worsening facial weakness accompanied by right ear pain, swelling, and drainage. Recently an outside facility prescribed him a 10-day course of antibiotics and corticosteroids with no significant improvement in symptoms. Examination revealed right auricular proptosis, erythema, and edema of the postauricular skin, and an open purulent wound inferior to the auricle. Examination of the external ear canal showed edema and significant purulent otorrhea. The patient had been told he needed ear surgery four years prior, but he never wanted it because he was worried about getting facial paralysis. Figure 1 shows his temporal bone computed tomography (CT) scan.Figure 1: Non-contrast axial (horizontal) CT of the right temporal bone showing extensive cholesteatoma that has eroded through the cochlea, semicircular canals, and tegmen. Audiology, otolaryngology, hearing loss.Figure 2: Non-contrast coronal (vertical parallel to face) CT of the right temporal bone showing extensive cholesteatoma that has eroded through the mastoid cavity, cochlea, and tegmen. There is a small persistent aerated portion of the middle ear space with the remnant ossicular chain in place. Audiology, otolaryngology, hearing loss.Figure 3: Axial (horizontal) Technetium SPECT nuclear scan showing bony inflammation and osteoblastic activity in the right temporal bone. Audiology, otolaryngology, hearing loss.Figure 4: Axial (horizontal) MRI T1 post-gadolinium contrast scan demonstrating inflammation within the temporal bone and periauricular soft tissue. Audiology, otolaryngology, hearing loss.Figure 5: Coronal (vertical parallel to face) MRI T1 post-gadolinium contras scan demonstrating right dural enhancement. Audiology, otolaryngology, hearing loss.Figure 6: Coronal (vertical parallel to face) MRI HASTE sequence demonstrating diffusion restriction in the right mastoid and middle ear. This finding is consistent with cholesteatoma. Audiology, otolaryngology, hearing loss.DIAGNOSIS: INFECTED CHOLESTEATOMA WITH OSTEOMYELITIS The patient's presentation is concerning for either a malignant process or an infectious process. Malignancies can sometimes grow quickly to the point where the vascular supply to the tumor cannot grow as fast as the tumor is growing. This can cause portions of the tumor to become necrotic (dead). The necrotic tissue can then become secondarily infected and cause significant edema. Malignancies can also involve the facial nerve slowly and cause facial paralysis. In fact, a slow onset facial paralysis needs work up to rule out malignancy. The first step in the evaluation of this patient should include obtaining CT and MRI imaging. The CT (Fig. 1) showed significant destruction of the temporal bone and destruction of the bone separating the brain from the mastoid (posterior fossa plate and tegmen; Fig. 2). If a mass is seen, a biopsy could be obtained to further evaluate the mass. If drainage is present, a culture should be obtained for treatment of the infection. The MRI (Figs. 4, 5) showed significant soft tissue inflammation. The degree of bone loss seen on CT also made us concerned that there may be osteomyelitis present and a technetium 99 SPECT scan was obtained. The SPECT scan (Fig. 3) showed there is also osteomyelitis of the temporal bone present in addition to the cholesteatoma found on MRI (Fig. 6). Skull base osteomyelitis (often referred to as malignant otitis externa) is a serious, potentially life-threatening infection of the external ear and skull base. It most commonly affects immunocompromised individuals (especially diabetic patients) who cannot prevent infection from spreading from the ear canal into the temporal bone. After entering the temporal bone, the infection can then progress rapidly within the skull base, ultimately leading to intracranial complications. This patient was a bit unusual in that he was not immunocompromised but he had significant bony destruction in the temporal bone and the cholesteatoma had secondarily become infected leading to chronic osteomyelitis. Our patient exhibited symptoms of skull base osteomyelitis, including severe ear pain and otorrhea. However, these symptoms were also concerning for possible malignancy, which must be ruled out with biopsy and culture. Examination of patients with skull base osteomyelitis typically reveals purulent drainage with a swollen and tender external auditory canal. Facial nerve paralysis is commonly experienced by patients with skull base osteomyelitis, cholesteatoma, and malignancies of the temporal bone. In this patient, the paralysis is likely due to cholesteatoma and overlying osteomyelitis. In this patient, temporal bone CT scan showed an enhancing lesion involving the right temporal bone and bony destruction of the right mastoid air cells, external auditory canal, and inner ear structures, suggesting cholesteatoma with secondary skull base osteomyelitis (Figs. 1, 2). Nuclear imaging with technetium-99 bone scan showed increased uptake of radiotracer in the areas of destructive changes identified on CT (Fig. 3). MRI of the brain and IAC further confirmed cholesteatoma centered on the right temporal bone involving these same structures (Figs. 4, 5, 6). The gold standard in the diagnosis of skull base osteomyelitis is a technetium-99 bone scan, which involves injection and visualization of a radiotracer that detects osteoblastic activity and concentrates in areas of bone remodeling. To achieve a higher level of accuracy, a technetium-99 SPECT scan may also be performed to localize the infection. After starting aggressive antibiotic treatment and establishing the diagnosis of skull base osteomyelitis with a technetium-99 bone scan, a gallium-67 scan (which concentrates in areas of active inflammation) is obtained to allow the physician to monitor the response to treatment, resolution of the infection, and appearance of recurrent disease. Unfortunately, clinicians who might be less aware of skull base osteomyelitis may only obtain a gallium-67 scan without a technetium-99 bone scan. A gallium-67 scan alone cannot differentiate between the skin and soft tissue infection in simple otitis externa versus the bone infection of skull base osteomyelitis. Failure to accurately diagnose the condition can lead to overly aggressive treatment of soft tissue infections with intravenous antibiotics. Though not always present in patients with skull base osteomyelitis, fever and elevated white blood cell count were present in our patient, suggesting a systemic inflammatory response to the infection, likely due to his normal immune function. Laboratory tests generally ordered for patients with suspected infection include ESR and CRP, which are markers of inflammation and can be useful in monitoring treatment response. Long-term culture-guided antibiotic therapy is the mainstay of treatment for skull base osteomyelitis, and its duration is dependent on symptom resolution, laboratory tests, and repeat gallium-67 bone scans. Ultimately, our patient was diagnosed with right-sided cholesteatoma with secondary skull base osteomyelitis and abscess formation associated with Pseudomonas infection. In the absence of diabetes or an other immunosuppressing condition, it is unusual that this patient developed skull base osteomyelitis. However, his pre-existing cholesteatoma may have served as a nidus for infection and a route for infection to enter the temporal bone. Cholesteatomas can invade and erode the structures of the middle ear (the ossicular chain), the inner ear, and the surrounding bone of the skull (mastoid cavity, tegmen, and petrous apex). This patient was first treated with long-term IV antibiotics for skull base osteomyelitis and to settle the skin and soft tissue infection. However, surgical resection to clear all epidermal elements from the temporal bone is the definitive treatment of cholesteatoma. Because the cholesteatoma eroded the bone of the cochlea, this patient had significant hearing loss, and a translabyrinthine approach would be used, during which the cochlea could potentially be preserved. The plan of preserving the cochlea would allow the patient to have a cochlear implant in the future. Ironically, the patient avoided surgery four years prior due to a fear of facial paralysis. However, cholesteatoma can lead to facial paralysis if left unchecked. In experienced hands, the likelihood of facial paralysis from cholesteatoma surgery is much lower than if it were left to grow, especially in a young patient. BONUS ONLINE VIDEOS: VISUAL DIAGNOSIS Read this month's Clinical Consultation case, then watch the accompanying videos from Hamid R. Djalilian, MD, to review the patient's imaging for yourself. Video 1. Axial (horizontal) CT of the temporal bone showing significant destruction of the inner ear structures. Video 2. Coronal (vertical parallel to face) CT of temporal bone showing the destruction of the bone separating the brain from the ear (tegmen). Video 3. Technetium 99 SPECT (fused axial CT image with bone scan image) at the basal turn of the cochlea level showing inflammation in the entire right temporal bone. Video 4. MRI T1 Axial post-contrast MRI showing inflammation in temporal bone and soft tissues of the auricle and scalp. Video 5. MRI T1 coronal post gad showing enhancement of dura. Video 6. Coronal HASTE sequence MRI showing the hyperintense (bright) area in right temporal bone indicating cholesteatoma. Watch the patient videos online at thehearingjournal.com

Symptoms: Unilateral Hearing Loss and Facial Paresis

Non-invasive acoustic neuroma treatment via Gamma Knife stereotactic radiosurgery

Objective:To elucidate the clinical characteristics, surgical strategy, facial nerve repair methods and outcomes of facial nerve schwannomas（FNS）. Methods:The clinical data of patients with FNS treated between January 2010 and December 2018 at Sun Yat-sen Memorial Hospital of Sun Yat-sen University were retrospectively collected, including the sidedness of FNS, clinical manifestations, imaging data, the extent of tumor, clinical management, preoperative and postoperative facial nerve function. Results:The major clinical manifestations of the 32 patients with FNS were facial palsy（27, 84.4%）, hearing loss（27, 84.4%）, tinnitus（22, 68.8%）, ear mass（15, 46.9%）, and stuffy feeling in the ear（13, 40.6%） respectively. Thirty patients were preoperatively diagnosed with FNS and 2 patients were misdiagnosed. 31 patients underwent resection of FNS, except one patient who was selected for long-term follow-up observation. The choice of surgical approach was based on the location, extent and auditory function of the FNS involved as well as the patient's wishes. The surgical approach was decided based on the location and extent of the tumor: 9 patients were operated via the inferior temporal fossa type A（Fisch A） approach; 8 patients were operated via the mastoid approach; 7 patients were operated via the enlarged mastoid approach; 3 patients were operated via the combined mastoid-cranial middle fossa approach; 1 patient was operated via the cranial middle fossa approach; 3 patients were operated via the combined Fisch A-cranial middle fossa approach. 28 patients（87.5%） had FNS with multiple segments of facial nerve involved. The most involved segment was the vertical segment of the facial nerve（26, 81.3%）. 15 patients underwent facial nerve repair simultaneously, including 7 cases of auricular nerve-facial nerve graft and 8 cases of facial nerve-sublingual nerve anastomosis. 4 cases had improved facial nerve function after auricular nerve-facial nerve graft and 2 cases had improved function after facial nerve-sublingual nerve anastomosis. Among patients who underwent facial nerve repair,the best outcome was H-B Ⅲ. Conclusion:The patients with FNS mainly presented with facial palsy and hearing loss. Temporal bone CT and cranial MR plain & enhanced scan served well to confirm the diagnosis. The improvement rate of postoperative facial nerve function was significantly higher in patients who underwent nerve repair than in those who did not. Hence, facial nerve repair should be considered. Compared with facial nerve-sublingual nerve anastomosis, auricular major nerve-facial nerve graft might be a better choice for improving postoperative facial nerve function.

Symptom: Serous Middle Ear Effusion and Hearing Loss

Symptom: Dizziness with Ear Pressure

Objective: To elucidate the clinical behavior, causes of misdiagnosis, surgical management, and outcomes of facial nerve schwannomas (FNS). Methods: A retrospective review in Chinese People's Liberation Army General Hospital from January 1, 2002 to December 31, 2015 was carried out and evaluated 110 patients with FNS, including 50 males and 60 females, aged 16-67 years old. The appropriate surgical strategy was selected based on each patient's clinical manifestations, facial nerve function, and imaging characteristics. After surgery, patients received follow-up visits to assess their facial nerve functions, with the effect of treatment compared to the reality before surgery. The Kruskal-Wallis H test was used to distinguish between the pre- and post-operation facial nerve functions in patients who had different facial nerve functions before the operations. Results: 110 cases of FNS mainly presented with facial paralysis, hearing loss, tinnitus, otalgia, dizziness, and facial spasm. 20 of the cases were misdiagnosed as Bell's Palsy, 6 were mistaken for chronic otitis media/cholesteatoma with radical mastoidectomy, 3 were mistaken for Meniere's disease, 1 was misdiagnosed as petrous bone cholesteatoma, and 4 were mistaken for acoustic neuroma. 81.8 % (90/110) of the patients had multiple segments of the facial nerve, including the vertical segment of the facial nerve, accounting for 65.5% (72/110), followed by the labyrinthine/geniculate segment, for 61.8% (68/110), and the horizontal segment, for 55.5% (61/110). The appropriate surgical approaches were chosed based on the sizes and scopes of the tumors evaluated by imaging: transmastoid approach in 73 cases, translabyrinthe approach in 14 cases, middle cranial fossa approach in 13 cases, retrosigmoid approach in 3 cases, transmastoid-middle cranial fossa approach in 3 cases, and transmastoid-neck approach in 4 cases, with all the patients undergoing a total/subtotal resection of the tumor. Eighty-seven patients had their facial nerves reconstructed. Among them, 6 received facial nerve end-to-end anastomosis, 55 received great auricular nerve graft, and 26 were subjected to facial nerve-hypoglossal nerve anastomosis. Because of long histories, facial muscle atrophies, or other reasons, the remaining patients were not received facial nerve reconstruction. The House-Brackmann(H-B) grading scale was used to evaluate the facial nerve function pre- and post-operation. Patients with better facial nerve functions and shorter history of facial paralysis before operation would get relatively better facial nerve function. The before and after operation comparisons revealed that the recovery of the facial nerve functions in patients with H-B Ⅰ-Ⅲ was better than the improvement in patients with H-B Ⅳ-Ⅴ. The difference was statistically significant (Kruskal-Wallis H test, H=8.508, P<0.05). Conclusions: The diagnosis of patients with unknown facial paralysis, hearing loss, and tinnitus should take into account the possibility of FNS. CT and other imaging examinations of the temporal bone can avoid misdiagnosis and determine the tumor size and extent of lesions, as well as provide the basis for the choice of the surgical approach. After tumors have been completely resected, facial nerve reconstruction can be performed simultaneously, according to the defect of the nerve.

Objective:To summarize and analyze the effect of facial nerve decompression surgery for the treatment of Bell's palsy and Hunt syndrome. Methods:The clinical data of 65 patients with facial nerve palsy who underwent facial nerve decompression in our center from October 2015 to October 2022 were retrospectively analyzed, including 54 patients with Bell's palsy and 11 patients with Hunter syndrome. The degree of facial paralysis（HB grade） was evaluated before surgery, and ENoG, pure tone audiometry, temporal bone CT and other examinations were completed. All patients had facial palsy with HB grade V or above after conservative treatment for at least 1 month, and ENoG decreased by more than 90%. All patients underwent facial nerve decompression surgery through the transmastoid approach within 3 months after onset of symptoms. The recovery effect of facial nerve function after surgery in patients with Bell's palsy and Hunter syndrome was summarized and analyzed. In addition, 15 cases in group A（operated within 30-60 days after onset） and 50 cases in group B（operated within 61-90 days after onset） were grouped according to the course of the disease（the interval between onset of symptoms and surgery） to explore the effect of surgical timing on postoperative effect. Results:There was no significant difference between the two groups of patients with Chi-square test（P=0.54） in 42 patients（77.8%, 42/54） with Bell's palsy and 7 patients（63.6%, 7/11） in patients with Hunter syndrome who recovered to grade Ⅰ-Ⅱ. According to the course of the disease, 10 cases（66.7%, 10/15） in group A recovered to grade Ⅰ-Ⅱ after surgery. In group B, 39 patients（78.0%, 39/50） recovered to grade Ⅰ-Ⅱ after surgery, and there was no statistically significant difference between the two groups by Chi-square test（P=0.58）. Conclusion:Patients with Bell's palsy and Hunter syndrome can achieve good results after facial nerve decompression within 3 months of onset, and there is no significant difference in the surgical effect between the two types of patients.