Objective
This study aimed to present clinical features and outcomes of 10 patients with intratemporal facial nerve neurofibromas.
Methods
The ten patients underwent complete tumor removal, and nerve grafting was performed on 3 cases whose nerve integrity was sacrificed. They were followed up after surgery.
Results
All patients firstly complained of facial palsy, and progressive sensorineural hearing loss was also present in one case. Insidious onset of facial palsy was found in 6 of 10 cases (60.0%). There was multi-segment involvement in 5 of 10 cases (50.0%). During the follow-up of 4.2 ± 1.5 ys (range, 2–6 ys), 1 of 6 cases who successfully maintained nerve integrity recovered to Grade III, and the others remained or recovered to Grade V or even Grade VI. In contrast, all of the 3 cases with nerve grafting recovered from Grade VI to Grade III or Grade IV. No tumor recurrence was noted.
Conclusions
Facial nerve neurofibromas mainly presented with insidious facial palsy, and tended to affect multiple segments of facial nerve. It seemed that complete tumor removal and nerve grafting had better outcomes of facial nerve compared to complete tumor removal with nerve integrity preserved.
1
Introduction
Facial nerve neurofibromas (FNN) are most commonly located in the parotid gland, and FNN in temporal bone are scarcely reported . Intratemporal FNN are benign tumors, and there haven’t been any malignant cases reported before, possibly due to their rarity , although malignant transformation occurs in up to 15% of cases with neurofibromatosis type I .
In the paper, we present and discuss clinical characteristics and outcomes of 10 cases with intratemporal FNN.
2
Materials and methods
The medical records of 10 patients with intratemporal FNN managed in our hospital between January, 1998 and January, 2012 were retrospectively reviewed. Neurofibromatosis was excluded, and the diagnosis of FNN was histologically confirmed. There were 4 female and 6 male. Mean age was 28.3 ± 1.4ys (range, 17–62 ys). The tumors were all removed completely by either transmastoid approach or middle cranial fossa approach or middle cranial fossa combined with transmastoid approach. 3 patients underwent sural or greater auricular nerve graft, since nerve deficit was formed during tumor removal. They were followed up after surgery. Facial nerve function was assessed by House–Brackmann grading system . Their clinical characteristics and outcomes of facial nerve were analyzed and discussed in the paper.
2
Materials and methods
The medical records of 10 patients with intratemporal FNN managed in our hospital between January, 1998 and January, 2012 were retrospectively reviewed. Neurofibromatosis was excluded, and the diagnosis of FNN was histologically confirmed. There were 4 female and 6 male. Mean age was 28.3 ± 1.4ys (range, 17–62 ys). The tumors were all removed completely by either transmastoid approach or middle cranial fossa approach or middle cranial fossa combined with transmastoid approach. 3 patients underwent sural or greater auricular nerve graft, since nerve deficit was formed during tumor removal. They were followed up after surgery. Facial nerve function was assessed by House–Brackmann grading system . Their clinical characteristics and outcomes of facial nerve were analyzed and discussed in the paper.
3
Results
Summary of the 10 cases is listed in Table 1 . Facial palsy was firstly presented in all cases, accompanied by progressive sensorineural hearing loss in one case due to compression of auditory nerve in internal auditory canal. The onset of facial palsy was insidious in 6 of 10 cases (60.0%), and sudden in 4 of 10 cases (40.0%). Otalgia occurred in 3 of 10 cases (30.0%) during the early stage of facial palsy.
| No. | Symptoms | Onset | Course (ys) | Segments affected | PreFNF | PosFNF | Surgical approach | NG | FU (ys) |
|---|---|---|---|---|---|---|---|---|---|
| 1 | FP, otalgia | Sudden | 2 | GG, LS | V | III | MCF | No | 2 |
| 2 | FP, SNHL | Insidious | 8 | IAC, LS, GG, TS, MS | VI | IV | MCF, TM | Yes | 3 |
| 3 | FP | Insidious | 5 | GG | V | V | MCF | No | 4 |
| 4 | FP, otalgia | Sudden | 1 | MS | VI | V | TM | No | 5 |
| 5 | FP | Insidious | 3 | LS, GG, TS | VI | VI | MCF, TM | No | 6 |
| 6 | FP | Sudden | 2 | GG | VI | VI | MCF | No | 2 |
| 7 | FP, otalgia | Insidious | 10 | TS, MS, | VI | IV | TM | Yes | 5 |
| 8 | FP | Sudden | 2 | TS,MS | VI | VI | TM | No | 5 |
| 9 | FP | Insidious | 2 | TS | VI | III | TM | Yes | 6 |
| 10 | FP | Insidious | 1 | GG | VI | V | MCF | No | 4 |
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